quality improvement in neurology: child neurology...
TRANSCRIPT
SPECIAL ARTICLE LEVEL OF RECOMMENDATION
Quality improvement in neurology Childneurology quality measure setExecutive summary
Anup D Patel MD Anne T Berg PhD Lori Billinghurst MD MSc FRCPC Daniel Fain MD Erin Fecske MSN
RN CNRN CPNP Tim Feyma MD Zachary Grinspan MD MS Amy Houtrow MD PhD MPH
Sanjeev Kothare MD Gogi Kumar MD Erin Lee Migvis Monduy MD Diego Morita MD
Christina L Szperka MD MSCE M Cristina Victorio MD Ann Yeh MD and Jeffrey R Buchhalter MD PhD
Neurologyreg 2018901-7 doi101212WNL0000000000004806
Correspondence
American Academy of
Neurology
qualityaancom
Childhood neurologic disorders as a group include relatively common conditions such asmigraine (prevalence between 3 and 106 in children 3ndash15 years of age12) transient ticdisorders (TDs) (3)3 and specifically Tourette syndrome (TS) (08)3 and rarer disorderssuch as infantile spasms that may occur in only about 1200 infants each year in the UnitedStates These disorders account for a disproportionately higher number of emergency de-partment visits intensive care admissions deaths and higher costs when compared to otherchildhood illness4 Generally delivery of quality care should improve outcomes and result indecreased unnecessary utilization of health services4
Transition from pediatric to adult care is a focus of the American Academy of Pediatrics andother organizations5 The issues surrounding transition for adolescents with chronic illnesscannot all be addressed at the primary care level and need to be addressed in specialty care Dueto these factors quality measures are needed and represent a current gap in this field
In 2016 the American Academy of Neurology (AAN) and Child Neurology Society (CNS)formed the Child Neurology Work Group to review existing guidelines current evidence andgaps in care The goal of thisWorkGroup was to develop ameasurement set for child neurologythat promotes quality improvement and drives better outcomes for neurologically ill childrenQuality measures are not guidelines Quality measures use guidelines and other best practices toassess and document performance of quality care to promote practice improvement when gapsin care exist Quality measures use current guidelines consensus statements and otherstandards
The AAN and CNS developed these quality measures based on the belief that specialists shouldplay a major role in selecting and creating measures that will drive improvement and possibly beused in accountability programs The AAN and CNS formed the Work Group with repre-sentatives from professional associations and patient advocacy organizations to ensure inputfrom a diverse set of members of the health care team
In this executive summary we report on the quality measurement set developed by the WorkGroup (table) The full measurement set including specifications is available online (httplinkslwwcomWNLA83)
From Nationwide Childrenrsquos Hospital (ADP) Columbus OH Ann amp Robert H Lurie Childrenrsquos Hospital of Chicago (ATB) Chicago IL Childrenrsquos Hospital of Philadelphia (LB CLS)PA Spectrum Health Helen Devos Childrenrsquos Hospital (DF) Grand Rapids MI Childrenrsquos Mercy Hospital (EF) Mission KS Gillette Childrenrsquos Specialty Health Care (TF) St Paul MNWeill Cornell Medicine (ZG) New York NY University of Pittsburgh (AH) PA Cohen Childrens Medical Center (SK) New Hyde Park NY Dayton Childrenrsquos Hospital (GK) OHAmerican Academy of Neurology (EL) Minneapolis MN Neuro Network Partners at Nicklaus Childrenrsquos Hospital (MM) Miami FL Cincinnati Childrenrsquos Hospital Medical Center (DM) Akron Childrenrsquos Hospital (MCV) OH Hospital for Sick Children (AY) Toronto and University of Calgary (JRB) Canada
Go to NeurologyorgN for full disclosures Funding information and disclosures deemed relevant by the authors if any are provided at the end of the article
Approved by the AAN Quality and Safety Subcommittee on December 22 2016 the AAN Practice Committee on December 28 2016 the Child Neurology Board of Directors onJanuary 9 2017 and the AANI Board of Directors on January 3 2017
Copyright copy 2017 American Academy of Neurology 1
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
Published Ahead of Print on December 15 2017 as 101212WNL0000000000004806
Opportunities for improvementChild neurology focuses on pediatric diseases across theneurologic spectrum Opportunities for quality improvementwere identified for a variety of neurologic conditions Reviewof the literature revealed gaps in care and areas where qualitymeasures might be used to drive improvement in care Thismeasurement set focuses on a subset of neurologic illness seenin the pediatric population
Infantile spasmsInfantile spasms (IS) is a potentially devastating early-lifeelectroclinical syndrome that can lead to developmental re-gression intellectual disability and lifelong epilepsy6ndash11 Theprevalence of IS ranges from 2 to 35 per 10000 live births peryear and is often accompanied by neurodevelopmental re-gression and hypsarrhythmia on EEG recording12ndash14 Suc-cessful early treatment can protect ongoing development andlead to permanent remission First-line treatments include
adrenocorticotropic hormone (ACTH) high-dose predniso-lone and vigabatrin Numerous studies have documenteddelays in use of inadequate or inappropriate treatment15
despite evidence from randomized trials an AAN practiceparameter and other consensus statements Delayed di-agnosis and treatment can result in lasting deleterious deficitsin development and cognition
Status epilepticusStatus epilepticus (SE) is a neurologic emergency characterizedby unremitting seizures or seizures that recur without return tobaseline mental status Estimates indicate 50000 to 150000people in the United States have an episode of SE each year16
SE can be convulsive (eg seizures with tonic tonic-clonic orclonic features) or nonconvulsive (eg absence status) Asconvulsive SE is associated with higher morbidity and mortalityit has been the focus of guidelines for evaluation and manage-ment The most recent guideline defines SE in children as anyclinical or electrographic seizure lasting longer than 5minutes1617 This guideline was designed to lead to a substantialreduction in morbidity and mortality associated with SE
TS and TDTDs including TS are the most commonly diagnosedmovement disorders in childhood with prevalence rates
Table 2016 Child neurology measurement set
Clinical problem Measure description
Infantile spasms Percentage of patients receiving appropriate first-line treatment for infantile spasms
Epilepsy Percentage of patients who received appropriately and correctly dosed rescue seizure therapy for children withepilepsy
Refractory convulsive statusepilepticus
Percentage of patients who received the start of a third-line therapy for seizure cessation for refractory convulsivestatus epilepticus
Epilepsy Percentage of patients with epilepsy screened for neurodevelopmental or neuropsychological deficits
Tic disorder and Tourettesyndrome
Percentage of patients who were queried for psychological or behavioral comorbid conditions of tic disorder orTourette syndrome
Tic disorder or Tourettesyndrome
Percentage of patients whowere treated or referred for treatment for comorbid symptoms of tic disorder or Tourettesyndrome
Tic disorder or Tourettesyndrome
Percentage of patients who were counseled or referred for behavioral therapy for management of chronic ticdisorder or Tourette syndrome
Global developmental delay Percentage of patients who had genetic testing ordered for global developmental delay
Chronic headache Percentage of patients who have been counseled to seek psychological or biobehavioral interventions formanagement of chronic headache
Spasticity or dystonia Percentage of patients with spasticity or dystonia who were evaluated or referred or treated with botulinum toxin A
Transition to adult neurologycare
Percentage of patients who had a neurologic transition plan of care
GlossaryAAN = American Academy of Neurology ACTH = adrenocorticotropic hormone CDH = chronic daily headache CMA =chromosomal microarray CNS = Child Neurology Society GDD = global developmental delay IS = infantile spasms SE =status epilepticus TD = tic disorder TS = Tourette syndrome
Supplemental DataFull Measurement Set at
NPuborgvwx8qf
2 Neurology | Volume 90 Number 2 | January 9 2018 NeurologyorgN
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
estimated between 03 and 1 of the population1819 Theyoccur more frequently in boys than girls and are characterizedby repetitive stereotyped involuntary movements andvocalizations18ndash20 TDs often resolve in childhood but maypersist into later adult life20 TS and TDs are associated withpsychiatric conditions such as attention-deficithyperactivitydisorder obsessive-compulsive disorder oppositional defiantdisorder and mood disorders including depression and anx-iety Intervention for these comorbidities can lead to sub-stantial functional improvements in this population but therate at which screening occurs for these conditions is un-known In addition effective behavioral interventions to re-duce the frequency of tics and improve quality of life are likelyunderutilized
Headache and migraineHeadache in particular migraine is a common pediatricproblem worldwide and it can become a chronic and dis-abling disorder21 Overall 60 of children are at risk forheadache in their lifetime with female patients being affectedat a greater rate than male patients21 The prevalence formigraine for those under 20 years of age is 7721 Headachescan become very frequent and evolve into chronic dailyheadache (CDH) a clinical syndrome that continues to beused frequently and defined as ldquopain localized to the headoccurring 15 or more days per month for more than 3monthsrdquo22 Chronic migraine a form of CDH is estimated tooccur in up to 18 of adolescents aged 12ndash17 years23 Whilethere are many pharmacologic treatments available for mi-graine and other primary headache disorders pediatric clinicaltrials including the recent Children and Adolescent MigrainePrevention (CHAMP) study consistently show high placeboresponse rates highlighting the psychological aspects of thedisorder that can be addressed by nonpharmacologic strate-gies Cognitive-behavioral therapy when combined with am-itriptyline has shown to be more effective in reducingheadache frequency and migraine-related disability amongyouth aged 10ndash17 years24 Nonpharmacologic treatment ofheadache such as psychological interventions have beenshown to be as effective as first-line therapy but the frequencyof adoption of this as first-line therapy in clinical practice isunknown25
Cerebral palsyCerebral palsy represents a group of nonprogressive dis-orders due to brain injury that affect postural and motorcontrol resulting in activity limitations26 Estimates from theCDC suggest an incidence of 33 per 1000 live births perCDC data27 Reduction of spasticity and dystonia can resultin increased mobility and decreased pain Medical treat-ments for spasticity and dystonia include oral medicationsor IM injections of botulinum toxin Surgical treatmentsinclude surgical placement of an intrathecal baclofen pumpdeep brain stimulator and selective dorsal rhizotomyMeasures to improve functioning include physical occu-pational and speech therapies as well as the use of orthosesand adaptive equipment28
Global developmental delayGlobal developmental delay (GDD) is diagnosed in childrenwith significant delay in acquiring early childhood de-velopmental milestones in more than 2 domains motorspeech and language cognitive and socialadaptive behav-ior29 The diagnosis is not synonymous with intellectual dis-ability and puts a child at risk for long-term cognitiveimpairments and functional dependence In children withoutautism between 1 and 3 meet criteria for GDD29 Ap-proximately 40 of otherwise unexplained GDD is due togenetic and metabolic disorders rather than environmentalfactors3031 Genetic testing that establishes a specific diagnosishas a number of benefits for patients and families and canresult in important changes in management32
Transition to adult neurologyThe shortage of pediatric neurology providers and increasingnumber of pediatric neurology patients limits the ability of thehealth care system to retain adult patients in the pediatricsystem33 As patients age they have concomitant healthproblems that are more commonly seen in the adult pop-ulation The transition of adolescents with chronic neurologicdisorders from a pediatric to an adult neurologist seems to bea sensible solution though it is a significant challenge3435
Young people referred to adult practitioners from tertiarypediatric centers have more complex health problems Adultproviders report lower levels of confidence in caring for thesepatients36 Other barriers to the transition to adult care are theadolescent and family anxiety of leaving familiar care which isoften family-centered The adult setting is not always equip-ped to handle intellectual disabilities and difficult behaviorwhich is more common in patients with chronic neurologicconditions of pediatric onset33 The American Academy ofPediatrics has outlined a formal transition process to addressthe gaps that currently exist in practice37 A transition plan isessential in ensuring that this occurs
MethodsThe AAN and CNS formed a cross-specialty and multidisci-plinary expert Work Group of diverse key stakeholders fromphysician and nonphysician associations patient and care-giver advocacy organizations and payers Details of the fullmeasure development process are available online38 Theformation of the Work Group began with a nominationprocess from AAN and CNS The co-chairs and facilitatorsselected members from the pool of qualified specialists andexpert nominees The selection was based on the nomineesrsquoexperience in quality measures quality improvement andclinical activities The final Work Group (23) includedphysicians advanced practice providers physical therapistsand a parent advocate (listed at the end of the manuscript)
Initially Work Group members disclosed potential conflictsof interest and completed applications summarizing experi-ences and interests Work Group members were selected
NeurologyorgN Neurology | Volume 90 Number 2 | January 9 2018 3
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
based on experience in various disciplines of child neurologyquality improvement and quality measurement
The measure development process included the following(1) evidence-based literature search (2) establishing a multi-disciplinary Work Group adhering to the AAN conflict ofinterest policy (3) drafting candidate measures and technicalspecifications (4) convening the Work Group in person toreview candidate measures (5) refining and discussing thecandidate measures (6) soliciting public comments on ap-proved measures during a 30-day period (7) refining the finalmeasures according to input received during the publiccomment period and corresponding technical specificationsand (8) obtaining approvals from the Work Group AANQuality and Safety Subcommittee AAN Practice CommitteeAAN Institute Board of Directors and CNS Board ofDirectors
TheWork Group sought to develop evidence-based measuresto support the delivery of high-quality care and to improvepatient outcomes The co-chairs and facilitators guided bya medical librarian conducted a comprehensive literaturesearch identifying 7840 abstracts relevant to the potentialmeasures including 22 clinical practice guidelines Followingthe development of draft measure concepts during the in-personmeeting a public comment period resulted in over 300comments from 60 individuals which drove conceptrefinement
The AAN and CNS plan to provide resources to update thesemeasures every 3 years which provides a working frameworkfor measurement rather than a long-term mandate
Prior to theWork Groupmeeting leadership put forth a set ofcandidate measures for Work Group review and discussionCandidate measures that adapted disease-specific measures tochildren were deferred to future revisions of disease-specificmeasurement sets in order to avoid duplication The WorkGroup met on June 10 2016 reviewed 12 candidate meas-ures and selected and modified 11 of the candidate measuresfor further consideration These measures were further re-fined based on public comments The Work Group approvedthe final 11 measures for inclusion in the AANCNS 2017Child Neurology Quality Measurement Set (table)
ResultsChild Neurology Quality Measurement SetUltimately the Work Group identified 11 quality metrics forimplementation in the care of children with neurologicdisorders
Although rare infantile (epileptic) spasms are a source ofsignificant morbidity mortality and resource expenditure inearly life There is controversy over what antiseizure medi-cation to use first-line but there is good evidence that ACTH
prednisolone or vigabatrin are all reasonable choices39ndash42
Literature suggests that prompt treatment can potentiallylessen the morbidity associated with IS and supports urgencyin initiating appropriate therapy While the precise criticalperiod of the intervention is unknown analysis of time totreatment in one large randomized trial showed that treat-ment initiation beyond 7 days after diagnosis was associatedwith worse developmental outcome at 4 years This suggeststhat initiation of therapy by 7 days may be key to improveddevelopmental outcomes64344
It has been more than a decade since the definition of SE wasnarrowed from a clinical seizure lasting longer than 30 to 5minutes45 Human and animal literature indicates that thesooner prolonged seizures are treated the greater the likeli-hood of termination Thus rescue therapy for children withconvulsive seizures with an appropriate medication at a dosethat is recommended to be effective should be provided forthe prehospital setting The intent is to reduce the morbidityand mortality that can accompany prolonged convulsiveseizures
Refractory SE is currently defined as seizures not responsiveto first- and second-line antiseizure drugs that include onenonbenzodiazepine and typically involves seizures lastinglonger than 30 minutes Although there is a paucity of for-mally conducted trials that assess timing of administration andhead-to-head trials of different agents consensus suggests thatthird-line medications with potential to treat refractory SEshould be administered within 60 minutes of onset in thehospital or arrival in the emergency department16
Children with epilepsy can have a variety of associated neu-rodevelopmental (eg specific learning) and neurobehavioral(eg attention-deficithyperactivity disorder autism spec-trum disorder) disabilities These can adversely affect qualityof life as much as or more than the actual epilepsy syndromeIt has been suggested that rather than comorbidities theseneurodevelopmental and behavioral difficulties are an integralpart of epilepsy Routine use of validated screening tools forneurodevelopmental and neurobehavioral abnormalities canallow for earlier diagnosis and improved outcomes Use ofthese instruments should not add substantially to theresources required for the care of these children with epilepsy
The most common movement disorder seen by child neu-rologists is TD which is seen transiently in childhood andincludes TS Although many tics of childhood are self-limiting common comorbidities include attention-deficithyperactivity disorder oppositional defiant disorderobsessive-compulsive disorder and mood disorders like anx-iety and depression As is the case for the epilepsies thesecomorbid conditions can be extremely debilitating for thechild and family Evaluating for these conditions is possible byasking relatively simple questions in the outpatient settingwith subsequent referral for more precise diagnosis andtreatment as needed The management of tic-related
4 Neurology | Volume 90 Number 2 | January 9 2018 NeurologyorgN
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
comorbidities can be performed by the child neurologist withappropriate pharmacotherapy or by referral to another careprovider like a child psychologist or psychiatrist Frequentlymedications can often be avoided as behavioral therapy forchronic TD and TS can be as effective as pharmacotherapy inreducing the frequency of tics and improving quality of lifeSeveral behavioral therapy approaches can be performed in anoffice setting by a child neurologist or by referral to a providerwho specializes in these treatment paradigms The type oftherapy chosen should be tailored to the needs of the child aswell as her or his ability to cooperate based upon level ofintellectual development and behavioral competence
Despite a variety of pharmacologic options migraine canbecome a chronic and debilitating condition in childhoodPsychological interventions (cognitive-behavioral therapybiofeedback or relaxation therapy) have been demonstratedto provide significant symptom relief and improve quality oflife
Spasticity and dystonia when localized or segmental can havemorbidity including pain and limitation of movement whichcan interfere with hygienecare and with the implementationof other therapies such as orthoses The care of children withspasticity and dystonia is best accomplished by a team ofindividuals to provide multifaceted treatment approachestailored to the needs of the patient These patients should beevaluated (or referred for evaluation) to a care provider ex-perienced in the use of botulinum A toxin which has beendemonstrated to be effective in reducing spasticity and dys-tonia In addition to symptom relief early use of this treat-ment modality can allow for improved function duringa critical period of motor development
GDD is a common problem that is referred to the pediatricneurologist The lack of a definitive diagnosis can delay onsetof therapies for treatable conditions therapy affect discussionrelated to prognosis and genetic counseling and frequentlyleads to unnecessary diagnostic testing The latter can beinvasive (eg lumbar puncture muscle biopsy) expensiveand lead to additional testing that may not be relevant In thiscontext it is reasonable to perform chromosomal microarray(CMA) as first-line testing Although CMA has sensitivity andspecificity limitations (like all other tests) it is the currentgateway to genetic diagnoses of GDD
Preparing patients and families for transition to adult care isone of the most important tasks to be performed in thelongitudinal care of a child with a chronic neurologic dis-order A variety of factors can influence creation and un-derstanding of this care plan including the developmentalstatus of the child as well as the need for and availability ofongoing neurologic care by an adult provider Neverthelessguidelines suggest that a written plan is in the best interest ofthe patient and the written plan should cover the medicalemotional social legal and financial needs associated withtransition to adult life
DiscussionThese quality measures represent a starting point for measuredevelopment in child neurology They are designed to allowchild neurologists to measure performance in areas wherethere are potential gaps in care Further work will be neededto identify and address other existing gaps in care The WorkGroup recognizes that resource gaps exist and may need to beaddressed to ensure success in complying with these qualitymeasures and ongoing advancement of best practices Theproposed quality measures are not intended to be used asproof of failure to provide optimal care when local resourcesmay not be able to accommodate The idea is not for thesemeasures to be used as guidelines or for the purposes ofpunishment for low performance Each child neurology pro-vider should take these measures and evaluate their currentbaseline Then by setting a goal for improvement of carequality improvement methodology can be implemented toimprove the outcomes and lives of patients with pediatricneurologic illnesses
Author contributionsDr Berg and Dr Billinghurst contributed to acquisition ofdata analysis or interpretation of data and critical revisionof the manuscript for important intellectual contentDr Buchhalter contributed to study concept and design ac-quisition of data analysis or interpretation of data draftingrevising the manuscript critical revision of the manuscript forimportant intellectual content and study supervisionDr Fain E Fecske Dr Feyma Dr Grinspan Dr HoutrowDr Kothare and Dr Kumar contributed to acquisition ofdata analysis or interpretation of data and critical revision ofthe manuscript for important intellectual content E Leecontributed to study concept and design acquisition of dataanalysis or interpretation of data draftingrevising the man-uscript critical revision of the manuscript for important in-tellectual content and study supervision Dr Monduy and DrMorita contributed to acquisition of data analysis or in-terpretation of data and critical revision of the manuscript forimportant intellectual content Dr Patel contributed to studyconcept and design acquisition of data analysis or in-terpretation of data draftingrevising the manuscript criticalrevision of the manuscript for important intellectual contentand study supervision Dr Szperka contributed to acquisitionof data analysis or interpretation of data and critical revisionof the manuscript for important intellectual content DrVictorio contributed to study concept and design acquisitionof data analysis or interpretation of data draftingrevising themanuscript critical revision of the manuscript for importantintellectual content and study supervision Dr Yeh contrib-uted to acquisition of data analysis or interpretation of dataand critical revision of the manuscript for important in-tellectual content
AcknowledgmentThe authors thank the Child Neurology Quality Measure-ment Set Work Group members for their dedication time
NeurologyorgN Neurology | Volume 90 Number 2 | January 9 2018 5
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
energy contributions and work that supported the de-velopment of this article Jeffrey Buchhalter MD FAAN(Chair Child Neurology Society) Anup Patel MD FAAN(Chair American Academy of Neurology) Karen Ballaban-Gil MD (American Academy of Neurology) Anne BergPhD (American Academy of Neurology) Daniel Fain MDFAAN (American Academy of Neurology) Cynthia KeatorMD (American Academy of Neurology) Sanjeev KothareMD (American Academy of Neurology) Gogi Kumar MD(American Academy of Neurology) Migvis MonduyMD (American Academy of Neurology) Diego Morita MD(American Academy of Neurology) Zachary Grinspan MDMS (American Academy of Pediatrics Child NeurologySociety) Erin Fecske MSN RN CNRN CPNP (AmericanAssociation of Neuroscience Nurses) Amy Houtrow MDPhD MPH (American Academy of Physical Medicine andRehabilitation) Kevin Chapman MD (American EpilepsySociety) Christina Szperka MD (American Headache Soci-ety) Lynn Jeffries PT DPT PhD PCS (American PhysicalTherapy Association) Tim Feyma MD (Child NeurologySociety) Kiran Maski MD (Child Neurology Society) LisaMeunier (Epilepsy Foundation) Lori Billinghurst MD MScFRCPC (independent) Ann Yeh MD (independent) DavidMichelsonMD (Facilitator AmericanAcademy ofNeurology)M Cristina Victorio MD (Facilitator American Academyof Neurology) Amy Bennett JD (American Academy ofNeurology staff) Gina Gjorvad (American Academy ofNeurology staff) Erin Lee (American Academy of Neurologystaff) Becky Schierman MPH (American Academy ofNeurology staff)
Study fundingNo targeted funding reported
DisclosureA Patel receives research grants from the Pediatric EpilepsyResearch Foundation Upsher-Smith GW Pharmaceuticalsand LivaNova He consults for GW Pharmaceuticals andLivaNova He is on a scientific advisory board for UCBPharma and Supernus A Berg and L Billinghurst report nodisclosures relevant to the manuscript D Fain is a sub-investigator for the following study as of September 6 2016 ARandomized Double-Blind Placebo-Controlled Study toEvaluate the Efficacy and Safety of Sage-547 Injection in theTreatment of Subjects with Super-Refractory Status Epi-lepticus Protocol number 547-SSE-301 E Fecske andT Feyma report no disclosures relevant to the manuscriptZ Grinspan receives funding from the Centers for DiseaseControl and Prevention the Pediatric Epilepsy ResearchFoundation and the Nanette Laitman Clinical Scholars Pro-gram A Houtrow S Kothare and G Kumar report no dis-closures relevant to the manuscript E Lee is an employee ofthe American Academy of Neurology M Monduy andD Morita report no disclosures relevant to the manuscriptC Szperka received funding from Pfizer andMiles for MigraineM Victorio reports no disclosures relevant to themanuscript AYeh receives research funding from the NMSS CMSC IORM
SCN CBMH Chase an Idea SickKids Foundation Rare Dis-eases Foundation MS Scientific Foundation (Canada)MSSociety McLaughlin Centre and Mario Battaglia FoundationShe has received unrestricted funds to run an educationalsymposium from theGuthy Jackson Charitable Foundation andTeva She has served on a scientific advisory board for neuro-toxicity for Juno She serves as a relapse adjudicator for ACI Shehas received honorarium for a talk from Novartis J Buchhalteris on the Scientific Advisory Board of the Charlie FoundationHe receives research funding from the Alberta ChildrenrsquosHospital Research Institute and the Department of PediatricsUniversity of Calgary Alberta Canada Go to NeurologyorgNfor full disclosures
Received May 17 2017 Accepted in final form September 25 2017
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3Sndash10S2 Lipton RB Bigal ME Migraine epidemiology impact and risk factors for pro-
gression Headache 200545(suppl 1)S3ndashS133 Knight T Steeves T Day L Lowerison M Jette N Pringsheim T Prevalence of tic
disorders a systematic review and meta-analysis Pediatr Neurol 20124777ndash904 Moreau JF Fink EL Hartman ME et al Hospitalizations of children with neurologic
disorders in the United States Pediatr Crit Care Med 201314801ndash8105 American Academy of Pediatrics Supporting the health care transition from ado-
lescence to adulthood in the medical home Pediatrics 2011128182ndash2006 Kivity S Lerman P Ariel R Danziger Y Mimouni M Shinnar S Long-term cognitive
outcomes of a cohort of children with cryptogenic infantile spasms treated with high-dose adrenocorticotropic hormone Epilepsia 200445255ndash262
7 Eisermann MM DeLaRaillere A Dellatolas G et al Infantile spasms in Down syn-drome effects of delayed anticonvulsive treatment Epilepsy Res 20035521ndash27
8 Goh S Kwiatkowski DJ Dorer DJ Thiele EA Infantile spasms and intellectualoutcomes in children with tuberous sclerosis complex Neurology 200565235ndash238
9 Riikonen R Long-term outcome in children with infantile spasms treated with vig-abatrin a cohort of 180 patients Epilepsia 201556807ndash809
10 Riikonen R A long-term follow-up study of 214 children with the syndrome ofinfantile spasms Neuropediatrics 19821314ndash23
11 Riikonen R Long-term outcome ofWest syndrome a study of adults with a history ofinfantile spasms Epilepsia 199637367ndash372
12 Widjaja E Go C McCoy B Snead O Neurodevelopmental outcome of infantilespasms a systematic review and meta-analysis Epilepsy Res 2015109155ndash162
13 Luthvigsson P Olafsson E Sigurdardottir S Hauser WA Epidemiologic features ofinfantile spasms in Iceland Epilepsia 199435802ndash805
14 Riikonen R Epidemiological data of West syndrome in Finland Brain Dev 200123539ndash541
15 Knupp KG Coryell J Nickels KC et al Response to treatment in a prospectivenational infantile spasms cohort Ann Neurol 201679475ndash484
16 Glauser T Shinnar S Gloss D et al Evidence-based guideline treatment of con-vulsive status epilepticus in children and adults report of the guideline committee ofthe American Epilepsy Society Epilepsy Curr 20161648ndash61
17 Brophy GM Bell R Claassen J et al Guidelines for the evaluation andmanagement ofstatus epilepticus Neurocrit Care 2012173ndash23
18 Cath D Hedderly T Ludolph A et al European clinical guidelines for Tourettesyndrome and other tic disorders part I assessment Eur Child Adolesc Psychiatry201120155ndash171
19 Knight T Steeves T Day L et al Prevalence of tic disorders a systematic review andmeta-analysis Pediatr Neurol 20124777ndash90
20 Tourette Syndrome Fact Sheet NIH Available at nindsnihgovdisorderstourettedetail_tourettehtm Accessed April 20 2016
21 Abu-Arafeh I Razak S Sivaraman B Graham C Prevalence of headache and migrainein children and adolescents a systematic review of population-based studies DevMedChild Neurol 2010521088ndash1097
22 Chiappedi M Mensi MM Termine C Balottin U Psychological therapy in adoles-cents with chronic daily headache Curr Pain Headache Rep 2016203
23 Lipton RB Nanack A Ricci JA et al Prevalence and burden of chronic migraine inadolescents results of the chronic daily headache in adolescents study (C-dAS)Headache 201151693ndash706
24 Powers SW Kashikar-Zuck SM Allen JR et al Cognitive behavioral therapy plusamitriptyline for chronic migraine in children and adolescents a randomized clinicaltrial JAMA 20133102622ndash2630
25 Faedda N Cerutti R Verdecchia P et al Behavioral management of headache inchildren and adolescents J Headache Pain 20161780
26 Bax M Goldstein M Rosenbaum P et al Executive Committee for the Definition ofCerebral Palsy Proposed definition and classification of cerebral palsy April 2005Dev Med Child Neurol 200547571ndash576
6 Neurology | Volume 90 Number 2 | January 9 2018 NeurologyorgN
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
27 Cerebral Palsy Fact Sheet NIH Available at nindsnihgovdisorderscerebral_palsydetail_cerebral_palsyhtm3104_2 Accessed September 3 2017
28 Shamsoddini A Amirsalari S Hollisaz MT Rahimnia A Khatibi-Aghda A Man-agement of spasticity in children with cerebral palsy Iranian J Pediatr 201424345ndash351
29 Michelson D Shevell M Sheer E et al Evidence report genetic and metabolic testingon children with global developmental delay Neurology 2011771629ndash1635
30 Kaufman L Ayub M Vincent JB The genetic basis of non-syndromic intellectualdisability a review J Neurodev Disord 20102182ndash209
31 Rauch A Hoyer J Guth S et al Diagnostic yield of various genetic approaches inpatients with unexplained developmental delay or mental retardation Am J MedGenet A 20061402063ndash2074
32 Riggs E Wain K Riethmaier D et al Chromosomal microarray impacts clinicalmanagement Clin Genet 201385147ndash153
33 Camfield P Gibson P Douglass L Strategies for transitioning to adult care for youthwith Lennox-Gastaut syndrome and related disorders Epilepsia 20115221ndash27
34 Brown L Camfield P Capers M et al The neurologistrsquos role in supporting transitionto adult health care Neurology 2016871ndash6
35 Camfield P Camfield C Transition to adult care for children with chronic neuro-logical disorders Ann Neurol 201169437ndash444
36 Borlot F Tellez-Zenteno JF Allen A Ali A Snead OC III Andrade DM Epilepsytransition challenges of caring for adults with childhood-onset seizures Epilepsia2014551659ndash1666
37 American Academy of Pediatrics Clinical report supporting the health care transitionfrom adolescence to adulthood in the medical home Pediatrics 2011128182ndash200
38 American Academy of Neurology Quality measurement manual 2014 Update Availableat aancomuploadedFilesWebsite_Library_AssetsDocuments3Practice_Manage-ment2Quality_Improvement1Quality_Measures2About_Quality_Measures20152002201120Process20Manual20Finalpdf Accessed March 9 2017
39 Go C Mackay M Weiss S et al Evidence-based guideline update medical treatmentof infantile spasms Neurology 2012781974ndash1980
40 Jones K Boyd J Go C et al Vigabatrin in the first line treatment of infantile spasmsEpilepsy Currents 201515533ndash534
41 Jones K Go C ACTH vs prednisolone in the treatment of infantile spasms postvigabatrin failure Epilepsy Currents 201414447ndash448
42 Jones K Go C Boyd J et al Vigabatrin as first-line treatment for infantile spasms notrelated to tuberous sclerosis complex Pediatr Neurol 201553141ndash145
43 Chapman KE Specchio N Shinnar S Holmes GL Seizing control of epileptic activitycan improve outcome Epilepsia 2015561482ndash1485
44 OrsquoCallaghan FJ Lux AL Darke K et al The effect of lead time to treatment andof age of onset on developmental outcome at 4 years in infantile spasms evi-dence from the United Kingdom Infantile Spasms Study Epilepsia 2011521359ndash1364
45 Trinka E Cock H Hesdorffer D et al A definition and classification of status epi-lepticus report of the ILAE Task Force on Classification of Status Epilepticus Epi-lepsia 2015561515ndash1523
NeurologyorgN Neurology | Volume 90 Number 2 | January 9 2018 7
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
DOI 101212WNL0000000000004806 published online December 15 2017Neurology
Anup D Patel Anne T Berg Lori Billinghurst et al summary
Quality improvement in neurology Child neurology quality measure set Executive
This information is current as of December 15 2017
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httpnneurologyorgcgicollectionall_health_services_researchAll Health Services Researchfollowing collection(s) This article along with others on similar topics appears in the
Permissions amp Licensing
httpnneurologyorgmiscaboutxhtmlpermissionsits entirety can be found online atInformation about reproducing this article in parts (figurestables) or in
Reprints
httpnneurologyorgmiscaddirxhtmlreprintsusInformation about ordering reprints can be found online
Neurology All rights reserved Print ISSN 0028-3878 Online ISSN 1526-632X1951 it is now a weekly with 48 issues per year Copyright Copyright copy 2017 American Academy of
reg is the official journal of the American Academy of Neurology Published continuously sinceNeurology
Opportunities for improvementChild neurology focuses on pediatric diseases across theneurologic spectrum Opportunities for quality improvementwere identified for a variety of neurologic conditions Reviewof the literature revealed gaps in care and areas where qualitymeasures might be used to drive improvement in care Thismeasurement set focuses on a subset of neurologic illness seenin the pediatric population
Infantile spasmsInfantile spasms (IS) is a potentially devastating early-lifeelectroclinical syndrome that can lead to developmental re-gression intellectual disability and lifelong epilepsy6ndash11 Theprevalence of IS ranges from 2 to 35 per 10000 live births peryear and is often accompanied by neurodevelopmental re-gression and hypsarrhythmia on EEG recording12ndash14 Suc-cessful early treatment can protect ongoing development andlead to permanent remission First-line treatments include
adrenocorticotropic hormone (ACTH) high-dose predniso-lone and vigabatrin Numerous studies have documenteddelays in use of inadequate or inappropriate treatment15
despite evidence from randomized trials an AAN practiceparameter and other consensus statements Delayed di-agnosis and treatment can result in lasting deleterious deficitsin development and cognition
Status epilepticusStatus epilepticus (SE) is a neurologic emergency characterizedby unremitting seizures or seizures that recur without return tobaseline mental status Estimates indicate 50000 to 150000people in the United States have an episode of SE each year16
SE can be convulsive (eg seizures with tonic tonic-clonic orclonic features) or nonconvulsive (eg absence status) Asconvulsive SE is associated with higher morbidity and mortalityit has been the focus of guidelines for evaluation and manage-ment The most recent guideline defines SE in children as anyclinical or electrographic seizure lasting longer than 5minutes1617 This guideline was designed to lead to a substantialreduction in morbidity and mortality associated with SE
TS and TDTDs including TS are the most commonly diagnosedmovement disorders in childhood with prevalence rates
Table 2016 Child neurology measurement set
Clinical problem Measure description
Infantile spasms Percentage of patients receiving appropriate first-line treatment for infantile spasms
Epilepsy Percentage of patients who received appropriately and correctly dosed rescue seizure therapy for children withepilepsy
Refractory convulsive statusepilepticus
Percentage of patients who received the start of a third-line therapy for seizure cessation for refractory convulsivestatus epilepticus
Epilepsy Percentage of patients with epilepsy screened for neurodevelopmental or neuropsychological deficits
Tic disorder and Tourettesyndrome
Percentage of patients who were queried for psychological or behavioral comorbid conditions of tic disorder orTourette syndrome
Tic disorder or Tourettesyndrome
Percentage of patients whowere treated or referred for treatment for comorbid symptoms of tic disorder or Tourettesyndrome
Tic disorder or Tourettesyndrome
Percentage of patients who were counseled or referred for behavioral therapy for management of chronic ticdisorder or Tourette syndrome
Global developmental delay Percentage of patients who had genetic testing ordered for global developmental delay
Chronic headache Percentage of patients who have been counseled to seek psychological or biobehavioral interventions formanagement of chronic headache
Spasticity or dystonia Percentage of patients with spasticity or dystonia who were evaluated or referred or treated with botulinum toxin A
Transition to adult neurologycare
Percentage of patients who had a neurologic transition plan of care
GlossaryAAN = American Academy of Neurology ACTH = adrenocorticotropic hormone CDH = chronic daily headache CMA =chromosomal microarray CNS = Child Neurology Society GDD = global developmental delay IS = infantile spasms SE =status epilepticus TD = tic disorder TS = Tourette syndrome
Supplemental DataFull Measurement Set at
NPuborgvwx8qf
2 Neurology | Volume 90 Number 2 | January 9 2018 NeurologyorgN
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
estimated between 03 and 1 of the population1819 Theyoccur more frequently in boys than girls and are characterizedby repetitive stereotyped involuntary movements andvocalizations18ndash20 TDs often resolve in childhood but maypersist into later adult life20 TS and TDs are associated withpsychiatric conditions such as attention-deficithyperactivitydisorder obsessive-compulsive disorder oppositional defiantdisorder and mood disorders including depression and anx-iety Intervention for these comorbidities can lead to sub-stantial functional improvements in this population but therate at which screening occurs for these conditions is un-known In addition effective behavioral interventions to re-duce the frequency of tics and improve quality of life are likelyunderutilized
Headache and migraineHeadache in particular migraine is a common pediatricproblem worldwide and it can become a chronic and dis-abling disorder21 Overall 60 of children are at risk forheadache in their lifetime with female patients being affectedat a greater rate than male patients21 The prevalence formigraine for those under 20 years of age is 7721 Headachescan become very frequent and evolve into chronic dailyheadache (CDH) a clinical syndrome that continues to beused frequently and defined as ldquopain localized to the headoccurring 15 or more days per month for more than 3monthsrdquo22 Chronic migraine a form of CDH is estimated tooccur in up to 18 of adolescents aged 12ndash17 years23 Whilethere are many pharmacologic treatments available for mi-graine and other primary headache disorders pediatric clinicaltrials including the recent Children and Adolescent MigrainePrevention (CHAMP) study consistently show high placeboresponse rates highlighting the psychological aspects of thedisorder that can be addressed by nonpharmacologic strate-gies Cognitive-behavioral therapy when combined with am-itriptyline has shown to be more effective in reducingheadache frequency and migraine-related disability amongyouth aged 10ndash17 years24 Nonpharmacologic treatment ofheadache such as psychological interventions have beenshown to be as effective as first-line therapy but the frequencyof adoption of this as first-line therapy in clinical practice isunknown25
Cerebral palsyCerebral palsy represents a group of nonprogressive dis-orders due to brain injury that affect postural and motorcontrol resulting in activity limitations26 Estimates from theCDC suggest an incidence of 33 per 1000 live births perCDC data27 Reduction of spasticity and dystonia can resultin increased mobility and decreased pain Medical treat-ments for spasticity and dystonia include oral medicationsor IM injections of botulinum toxin Surgical treatmentsinclude surgical placement of an intrathecal baclofen pumpdeep brain stimulator and selective dorsal rhizotomyMeasures to improve functioning include physical occu-pational and speech therapies as well as the use of orthosesand adaptive equipment28
Global developmental delayGlobal developmental delay (GDD) is diagnosed in childrenwith significant delay in acquiring early childhood de-velopmental milestones in more than 2 domains motorspeech and language cognitive and socialadaptive behav-ior29 The diagnosis is not synonymous with intellectual dis-ability and puts a child at risk for long-term cognitiveimpairments and functional dependence In children withoutautism between 1 and 3 meet criteria for GDD29 Ap-proximately 40 of otherwise unexplained GDD is due togenetic and metabolic disorders rather than environmentalfactors3031 Genetic testing that establishes a specific diagnosishas a number of benefits for patients and families and canresult in important changes in management32
Transition to adult neurologyThe shortage of pediatric neurology providers and increasingnumber of pediatric neurology patients limits the ability of thehealth care system to retain adult patients in the pediatricsystem33 As patients age they have concomitant healthproblems that are more commonly seen in the adult pop-ulation The transition of adolescents with chronic neurologicdisorders from a pediatric to an adult neurologist seems to bea sensible solution though it is a significant challenge3435
Young people referred to adult practitioners from tertiarypediatric centers have more complex health problems Adultproviders report lower levels of confidence in caring for thesepatients36 Other barriers to the transition to adult care are theadolescent and family anxiety of leaving familiar care which isoften family-centered The adult setting is not always equip-ped to handle intellectual disabilities and difficult behaviorwhich is more common in patients with chronic neurologicconditions of pediatric onset33 The American Academy ofPediatrics has outlined a formal transition process to addressthe gaps that currently exist in practice37 A transition plan isessential in ensuring that this occurs
MethodsThe AAN and CNS formed a cross-specialty and multidisci-plinary expert Work Group of diverse key stakeholders fromphysician and nonphysician associations patient and care-giver advocacy organizations and payers Details of the fullmeasure development process are available online38 Theformation of the Work Group began with a nominationprocess from AAN and CNS The co-chairs and facilitatorsselected members from the pool of qualified specialists andexpert nominees The selection was based on the nomineesrsquoexperience in quality measures quality improvement andclinical activities The final Work Group (23) includedphysicians advanced practice providers physical therapistsand a parent advocate (listed at the end of the manuscript)
Initially Work Group members disclosed potential conflictsof interest and completed applications summarizing experi-ences and interests Work Group members were selected
NeurologyorgN Neurology | Volume 90 Number 2 | January 9 2018 3
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
based on experience in various disciplines of child neurologyquality improvement and quality measurement
The measure development process included the following(1) evidence-based literature search (2) establishing a multi-disciplinary Work Group adhering to the AAN conflict ofinterest policy (3) drafting candidate measures and technicalspecifications (4) convening the Work Group in person toreview candidate measures (5) refining and discussing thecandidate measures (6) soliciting public comments on ap-proved measures during a 30-day period (7) refining the finalmeasures according to input received during the publiccomment period and corresponding technical specificationsand (8) obtaining approvals from the Work Group AANQuality and Safety Subcommittee AAN Practice CommitteeAAN Institute Board of Directors and CNS Board ofDirectors
TheWork Group sought to develop evidence-based measuresto support the delivery of high-quality care and to improvepatient outcomes The co-chairs and facilitators guided bya medical librarian conducted a comprehensive literaturesearch identifying 7840 abstracts relevant to the potentialmeasures including 22 clinical practice guidelines Followingthe development of draft measure concepts during the in-personmeeting a public comment period resulted in over 300comments from 60 individuals which drove conceptrefinement
The AAN and CNS plan to provide resources to update thesemeasures every 3 years which provides a working frameworkfor measurement rather than a long-term mandate
Prior to theWork Groupmeeting leadership put forth a set ofcandidate measures for Work Group review and discussionCandidate measures that adapted disease-specific measures tochildren were deferred to future revisions of disease-specificmeasurement sets in order to avoid duplication The WorkGroup met on June 10 2016 reviewed 12 candidate meas-ures and selected and modified 11 of the candidate measuresfor further consideration These measures were further re-fined based on public comments The Work Group approvedthe final 11 measures for inclusion in the AANCNS 2017Child Neurology Quality Measurement Set (table)
ResultsChild Neurology Quality Measurement SetUltimately the Work Group identified 11 quality metrics forimplementation in the care of children with neurologicdisorders
Although rare infantile (epileptic) spasms are a source ofsignificant morbidity mortality and resource expenditure inearly life There is controversy over what antiseizure medi-cation to use first-line but there is good evidence that ACTH
prednisolone or vigabatrin are all reasonable choices39ndash42
Literature suggests that prompt treatment can potentiallylessen the morbidity associated with IS and supports urgencyin initiating appropriate therapy While the precise criticalperiod of the intervention is unknown analysis of time totreatment in one large randomized trial showed that treat-ment initiation beyond 7 days after diagnosis was associatedwith worse developmental outcome at 4 years This suggeststhat initiation of therapy by 7 days may be key to improveddevelopmental outcomes64344
It has been more than a decade since the definition of SE wasnarrowed from a clinical seizure lasting longer than 30 to 5minutes45 Human and animal literature indicates that thesooner prolonged seizures are treated the greater the likeli-hood of termination Thus rescue therapy for children withconvulsive seizures with an appropriate medication at a dosethat is recommended to be effective should be provided forthe prehospital setting The intent is to reduce the morbidityand mortality that can accompany prolonged convulsiveseizures
Refractory SE is currently defined as seizures not responsiveto first- and second-line antiseizure drugs that include onenonbenzodiazepine and typically involves seizures lastinglonger than 30 minutes Although there is a paucity of for-mally conducted trials that assess timing of administration andhead-to-head trials of different agents consensus suggests thatthird-line medications with potential to treat refractory SEshould be administered within 60 minutes of onset in thehospital or arrival in the emergency department16
Children with epilepsy can have a variety of associated neu-rodevelopmental (eg specific learning) and neurobehavioral(eg attention-deficithyperactivity disorder autism spec-trum disorder) disabilities These can adversely affect qualityof life as much as or more than the actual epilepsy syndromeIt has been suggested that rather than comorbidities theseneurodevelopmental and behavioral difficulties are an integralpart of epilepsy Routine use of validated screening tools forneurodevelopmental and neurobehavioral abnormalities canallow for earlier diagnosis and improved outcomes Use ofthese instruments should not add substantially to theresources required for the care of these children with epilepsy
The most common movement disorder seen by child neu-rologists is TD which is seen transiently in childhood andincludes TS Although many tics of childhood are self-limiting common comorbidities include attention-deficithyperactivity disorder oppositional defiant disorderobsessive-compulsive disorder and mood disorders like anx-iety and depression As is the case for the epilepsies thesecomorbid conditions can be extremely debilitating for thechild and family Evaluating for these conditions is possible byasking relatively simple questions in the outpatient settingwith subsequent referral for more precise diagnosis andtreatment as needed The management of tic-related
4 Neurology | Volume 90 Number 2 | January 9 2018 NeurologyorgN
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
comorbidities can be performed by the child neurologist withappropriate pharmacotherapy or by referral to another careprovider like a child psychologist or psychiatrist Frequentlymedications can often be avoided as behavioral therapy forchronic TD and TS can be as effective as pharmacotherapy inreducing the frequency of tics and improving quality of lifeSeveral behavioral therapy approaches can be performed in anoffice setting by a child neurologist or by referral to a providerwho specializes in these treatment paradigms The type oftherapy chosen should be tailored to the needs of the child aswell as her or his ability to cooperate based upon level ofintellectual development and behavioral competence
Despite a variety of pharmacologic options migraine canbecome a chronic and debilitating condition in childhoodPsychological interventions (cognitive-behavioral therapybiofeedback or relaxation therapy) have been demonstratedto provide significant symptom relief and improve quality oflife
Spasticity and dystonia when localized or segmental can havemorbidity including pain and limitation of movement whichcan interfere with hygienecare and with the implementationof other therapies such as orthoses The care of children withspasticity and dystonia is best accomplished by a team ofindividuals to provide multifaceted treatment approachestailored to the needs of the patient These patients should beevaluated (or referred for evaluation) to a care provider ex-perienced in the use of botulinum A toxin which has beendemonstrated to be effective in reducing spasticity and dys-tonia In addition to symptom relief early use of this treat-ment modality can allow for improved function duringa critical period of motor development
GDD is a common problem that is referred to the pediatricneurologist The lack of a definitive diagnosis can delay onsetof therapies for treatable conditions therapy affect discussionrelated to prognosis and genetic counseling and frequentlyleads to unnecessary diagnostic testing The latter can beinvasive (eg lumbar puncture muscle biopsy) expensiveand lead to additional testing that may not be relevant In thiscontext it is reasonable to perform chromosomal microarray(CMA) as first-line testing Although CMA has sensitivity andspecificity limitations (like all other tests) it is the currentgateway to genetic diagnoses of GDD
Preparing patients and families for transition to adult care isone of the most important tasks to be performed in thelongitudinal care of a child with a chronic neurologic dis-order A variety of factors can influence creation and un-derstanding of this care plan including the developmentalstatus of the child as well as the need for and availability ofongoing neurologic care by an adult provider Neverthelessguidelines suggest that a written plan is in the best interest ofthe patient and the written plan should cover the medicalemotional social legal and financial needs associated withtransition to adult life
DiscussionThese quality measures represent a starting point for measuredevelopment in child neurology They are designed to allowchild neurologists to measure performance in areas wherethere are potential gaps in care Further work will be neededto identify and address other existing gaps in care The WorkGroup recognizes that resource gaps exist and may need to beaddressed to ensure success in complying with these qualitymeasures and ongoing advancement of best practices Theproposed quality measures are not intended to be used asproof of failure to provide optimal care when local resourcesmay not be able to accommodate The idea is not for thesemeasures to be used as guidelines or for the purposes ofpunishment for low performance Each child neurology pro-vider should take these measures and evaluate their currentbaseline Then by setting a goal for improvement of carequality improvement methodology can be implemented toimprove the outcomes and lives of patients with pediatricneurologic illnesses
Author contributionsDr Berg and Dr Billinghurst contributed to acquisition ofdata analysis or interpretation of data and critical revisionof the manuscript for important intellectual contentDr Buchhalter contributed to study concept and design ac-quisition of data analysis or interpretation of data draftingrevising the manuscript critical revision of the manuscript forimportant intellectual content and study supervisionDr Fain E Fecske Dr Feyma Dr Grinspan Dr HoutrowDr Kothare and Dr Kumar contributed to acquisition ofdata analysis or interpretation of data and critical revision ofthe manuscript for important intellectual content E Leecontributed to study concept and design acquisition of dataanalysis or interpretation of data draftingrevising the man-uscript critical revision of the manuscript for important in-tellectual content and study supervision Dr Monduy and DrMorita contributed to acquisition of data analysis or in-terpretation of data and critical revision of the manuscript forimportant intellectual content Dr Patel contributed to studyconcept and design acquisition of data analysis or in-terpretation of data draftingrevising the manuscript criticalrevision of the manuscript for important intellectual contentand study supervision Dr Szperka contributed to acquisitionof data analysis or interpretation of data and critical revisionof the manuscript for important intellectual content DrVictorio contributed to study concept and design acquisitionof data analysis or interpretation of data draftingrevising themanuscript critical revision of the manuscript for importantintellectual content and study supervision Dr Yeh contrib-uted to acquisition of data analysis or interpretation of dataand critical revision of the manuscript for important in-tellectual content
AcknowledgmentThe authors thank the Child Neurology Quality Measure-ment Set Work Group members for their dedication time
NeurologyorgN Neurology | Volume 90 Number 2 | January 9 2018 5
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
energy contributions and work that supported the de-velopment of this article Jeffrey Buchhalter MD FAAN(Chair Child Neurology Society) Anup Patel MD FAAN(Chair American Academy of Neurology) Karen Ballaban-Gil MD (American Academy of Neurology) Anne BergPhD (American Academy of Neurology) Daniel Fain MDFAAN (American Academy of Neurology) Cynthia KeatorMD (American Academy of Neurology) Sanjeev KothareMD (American Academy of Neurology) Gogi Kumar MD(American Academy of Neurology) Migvis MonduyMD (American Academy of Neurology) Diego Morita MD(American Academy of Neurology) Zachary Grinspan MDMS (American Academy of Pediatrics Child NeurologySociety) Erin Fecske MSN RN CNRN CPNP (AmericanAssociation of Neuroscience Nurses) Amy Houtrow MDPhD MPH (American Academy of Physical Medicine andRehabilitation) Kevin Chapman MD (American EpilepsySociety) Christina Szperka MD (American Headache Soci-ety) Lynn Jeffries PT DPT PhD PCS (American PhysicalTherapy Association) Tim Feyma MD (Child NeurologySociety) Kiran Maski MD (Child Neurology Society) LisaMeunier (Epilepsy Foundation) Lori Billinghurst MD MScFRCPC (independent) Ann Yeh MD (independent) DavidMichelsonMD (Facilitator AmericanAcademy ofNeurology)M Cristina Victorio MD (Facilitator American Academyof Neurology) Amy Bennett JD (American Academy ofNeurology staff) Gina Gjorvad (American Academy ofNeurology staff) Erin Lee (American Academy of Neurologystaff) Becky Schierman MPH (American Academy ofNeurology staff)
Study fundingNo targeted funding reported
DisclosureA Patel receives research grants from the Pediatric EpilepsyResearch Foundation Upsher-Smith GW Pharmaceuticalsand LivaNova He consults for GW Pharmaceuticals andLivaNova He is on a scientific advisory board for UCBPharma and Supernus A Berg and L Billinghurst report nodisclosures relevant to the manuscript D Fain is a sub-investigator for the following study as of September 6 2016 ARandomized Double-Blind Placebo-Controlled Study toEvaluate the Efficacy and Safety of Sage-547 Injection in theTreatment of Subjects with Super-Refractory Status Epi-lepticus Protocol number 547-SSE-301 E Fecske andT Feyma report no disclosures relevant to the manuscriptZ Grinspan receives funding from the Centers for DiseaseControl and Prevention the Pediatric Epilepsy ResearchFoundation and the Nanette Laitman Clinical Scholars Pro-gram A Houtrow S Kothare and G Kumar report no dis-closures relevant to the manuscript E Lee is an employee ofthe American Academy of Neurology M Monduy andD Morita report no disclosures relevant to the manuscriptC Szperka received funding from Pfizer andMiles for MigraineM Victorio reports no disclosures relevant to themanuscript AYeh receives research funding from the NMSS CMSC IORM
SCN CBMH Chase an Idea SickKids Foundation Rare Dis-eases Foundation MS Scientific Foundation (Canada)MSSociety McLaughlin Centre and Mario Battaglia FoundationShe has received unrestricted funds to run an educationalsymposium from theGuthy Jackson Charitable Foundation andTeva She has served on a scientific advisory board for neuro-toxicity for Juno She serves as a relapse adjudicator for ACI Shehas received honorarium for a talk from Novartis J Buchhalteris on the Scientific Advisory Board of the Charlie FoundationHe receives research funding from the Alberta ChildrenrsquosHospital Research Institute and the Department of PediatricsUniversity of Calgary Alberta Canada Go to NeurologyorgNfor full disclosures
Received May 17 2017 Accepted in final form September 25 2017
References1 Lipton RB Bigal ME The epidemiology of migraine Am J Med 2005118(suppl 1)
3Sndash10S2 Lipton RB Bigal ME Migraine epidemiology impact and risk factors for pro-
gression Headache 200545(suppl 1)S3ndashS133 Knight T Steeves T Day L Lowerison M Jette N Pringsheim T Prevalence of tic
disorders a systematic review and meta-analysis Pediatr Neurol 20124777ndash904 Moreau JF Fink EL Hartman ME et al Hospitalizations of children with neurologic
disorders in the United States Pediatr Crit Care Med 201314801ndash8105 American Academy of Pediatrics Supporting the health care transition from ado-
lescence to adulthood in the medical home Pediatrics 2011128182ndash2006 Kivity S Lerman P Ariel R Danziger Y Mimouni M Shinnar S Long-term cognitive
outcomes of a cohort of children with cryptogenic infantile spasms treated with high-dose adrenocorticotropic hormone Epilepsia 200445255ndash262
7 Eisermann MM DeLaRaillere A Dellatolas G et al Infantile spasms in Down syn-drome effects of delayed anticonvulsive treatment Epilepsy Res 20035521ndash27
8 Goh S Kwiatkowski DJ Dorer DJ Thiele EA Infantile spasms and intellectualoutcomes in children with tuberous sclerosis complex Neurology 200565235ndash238
9 Riikonen R Long-term outcome in children with infantile spasms treated with vig-abatrin a cohort of 180 patients Epilepsia 201556807ndash809
10 Riikonen R A long-term follow-up study of 214 children with the syndrome ofinfantile spasms Neuropediatrics 19821314ndash23
11 Riikonen R Long-term outcome ofWest syndrome a study of adults with a history ofinfantile spasms Epilepsia 199637367ndash372
12 Widjaja E Go C McCoy B Snead O Neurodevelopmental outcome of infantilespasms a systematic review and meta-analysis Epilepsy Res 2015109155ndash162
13 Luthvigsson P Olafsson E Sigurdardottir S Hauser WA Epidemiologic features ofinfantile spasms in Iceland Epilepsia 199435802ndash805
14 Riikonen R Epidemiological data of West syndrome in Finland Brain Dev 200123539ndash541
15 Knupp KG Coryell J Nickels KC et al Response to treatment in a prospectivenational infantile spasms cohort Ann Neurol 201679475ndash484
16 Glauser T Shinnar S Gloss D et al Evidence-based guideline treatment of con-vulsive status epilepticus in children and adults report of the guideline committee ofthe American Epilepsy Society Epilepsy Curr 20161648ndash61
17 Brophy GM Bell R Claassen J et al Guidelines for the evaluation andmanagement ofstatus epilepticus Neurocrit Care 2012173ndash23
18 Cath D Hedderly T Ludolph A et al European clinical guidelines for Tourettesyndrome and other tic disorders part I assessment Eur Child Adolesc Psychiatry201120155ndash171
19 Knight T Steeves T Day L et al Prevalence of tic disorders a systematic review andmeta-analysis Pediatr Neurol 20124777ndash90
20 Tourette Syndrome Fact Sheet NIH Available at nindsnihgovdisorderstourettedetail_tourettehtm Accessed April 20 2016
21 Abu-Arafeh I Razak S Sivaraman B Graham C Prevalence of headache and migrainein children and adolescents a systematic review of population-based studies DevMedChild Neurol 2010521088ndash1097
22 Chiappedi M Mensi MM Termine C Balottin U Psychological therapy in adoles-cents with chronic daily headache Curr Pain Headache Rep 2016203
23 Lipton RB Nanack A Ricci JA et al Prevalence and burden of chronic migraine inadolescents results of the chronic daily headache in adolescents study (C-dAS)Headache 201151693ndash706
24 Powers SW Kashikar-Zuck SM Allen JR et al Cognitive behavioral therapy plusamitriptyline for chronic migraine in children and adolescents a randomized clinicaltrial JAMA 20133102622ndash2630
25 Faedda N Cerutti R Verdecchia P et al Behavioral management of headache inchildren and adolescents J Headache Pain 20161780
26 Bax M Goldstein M Rosenbaum P et al Executive Committee for the Definition ofCerebral Palsy Proposed definition and classification of cerebral palsy April 2005Dev Med Child Neurol 200547571ndash576
6 Neurology | Volume 90 Number 2 | January 9 2018 NeurologyorgN
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
27 Cerebral Palsy Fact Sheet NIH Available at nindsnihgovdisorderscerebral_palsydetail_cerebral_palsyhtm3104_2 Accessed September 3 2017
28 Shamsoddini A Amirsalari S Hollisaz MT Rahimnia A Khatibi-Aghda A Man-agement of spasticity in children with cerebral palsy Iranian J Pediatr 201424345ndash351
29 Michelson D Shevell M Sheer E et al Evidence report genetic and metabolic testingon children with global developmental delay Neurology 2011771629ndash1635
30 Kaufman L Ayub M Vincent JB The genetic basis of non-syndromic intellectualdisability a review J Neurodev Disord 20102182ndash209
31 Rauch A Hoyer J Guth S et al Diagnostic yield of various genetic approaches inpatients with unexplained developmental delay or mental retardation Am J MedGenet A 20061402063ndash2074
32 Riggs E Wain K Riethmaier D et al Chromosomal microarray impacts clinicalmanagement Clin Genet 201385147ndash153
33 Camfield P Gibson P Douglass L Strategies for transitioning to adult care for youthwith Lennox-Gastaut syndrome and related disorders Epilepsia 20115221ndash27
34 Brown L Camfield P Capers M et al The neurologistrsquos role in supporting transitionto adult health care Neurology 2016871ndash6
35 Camfield P Camfield C Transition to adult care for children with chronic neuro-logical disorders Ann Neurol 201169437ndash444
36 Borlot F Tellez-Zenteno JF Allen A Ali A Snead OC III Andrade DM Epilepsytransition challenges of caring for adults with childhood-onset seizures Epilepsia2014551659ndash1666
37 American Academy of Pediatrics Clinical report supporting the health care transitionfrom adolescence to adulthood in the medical home Pediatrics 2011128182ndash200
38 American Academy of Neurology Quality measurement manual 2014 Update Availableat aancomuploadedFilesWebsite_Library_AssetsDocuments3Practice_Manage-ment2Quality_Improvement1Quality_Measures2About_Quality_Measures20152002201120Process20Manual20Finalpdf Accessed March 9 2017
39 Go C Mackay M Weiss S et al Evidence-based guideline update medical treatmentof infantile spasms Neurology 2012781974ndash1980
40 Jones K Boyd J Go C et al Vigabatrin in the first line treatment of infantile spasmsEpilepsy Currents 201515533ndash534
41 Jones K Go C ACTH vs prednisolone in the treatment of infantile spasms postvigabatrin failure Epilepsy Currents 201414447ndash448
42 Jones K Go C Boyd J et al Vigabatrin as first-line treatment for infantile spasms notrelated to tuberous sclerosis complex Pediatr Neurol 201553141ndash145
43 Chapman KE Specchio N Shinnar S Holmes GL Seizing control of epileptic activitycan improve outcome Epilepsia 2015561482ndash1485
44 OrsquoCallaghan FJ Lux AL Darke K et al The effect of lead time to treatment andof age of onset on developmental outcome at 4 years in infantile spasms evi-dence from the United Kingdom Infantile Spasms Study Epilepsia 2011521359ndash1364
45 Trinka E Cock H Hesdorffer D et al A definition and classification of status epi-lepticus report of the ILAE Task Force on Classification of Status Epilepticus Epi-lepsia 2015561515ndash1523
NeurologyorgN Neurology | Volume 90 Number 2 | January 9 2018 7
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
DOI 101212WNL0000000000004806 published online December 15 2017Neurology
Anup D Patel Anne T Berg Lori Billinghurst et al summary
Quality improvement in neurology Child neurology quality measure set Executive
This information is current as of December 15 2017
ServicesUpdated Information amp
806fullhtmlhttpnneurologyorgcontentearly20171215WNL0000000000004including high resolution figures can be found at
Citations
806fullhtmlotherarticleshttpnneurologyorgcontentearly20171215WNL0000000000004This article has been cited by 1 HighWire-hosted articles
Subspecialty Collections
httpnneurologyorgcgicollectionall_pediatricAll Pediatric
httpnneurologyorgcgicollectionall_health_services_researchAll Health Services Researchfollowing collection(s) This article along with others on similar topics appears in the
Permissions amp Licensing
httpnneurologyorgmiscaboutxhtmlpermissionsits entirety can be found online atInformation about reproducing this article in parts (figurestables) or in
Reprints
httpnneurologyorgmiscaddirxhtmlreprintsusInformation about ordering reprints can be found online
Neurology All rights reserved Print ISSN 0028-3878 Online ISSN 1526-632X1951 it is now a weekly with 48 issues per year Copyright Copyright copy 2017 American Academy of
reg is the official journal of the American Academy of Neurology Published continuously sinceNeurology
estimated between 03 and 1 of the population1819 Theyoccur more frequently in boys than girls and are characterizedby repetitive stereotyped involuntary movements andvocalizations18ndash20 TDs often resolve in childhood but maypersist into later adult life20 TS and TDs are associated withpsychiatric conditions such as attention-deficithyperactivitydisorder obsessive-compulsive disorder oppositional defiantdisorder and mood disorders including depression and anx-iety Intervention for these comorbidities can lead to sub-stantial functional improvements in this population but therate at which screening occurs for these conditions is un-known In addition effective behavioral interventions to re-duce the frequency of tics and improve quality of life are likelyunderutilized
Headache and migraineHeadache in particular migraine is a common pediatricproblem worldwide and it can become a chronic and dis-abling disorder21 Overall 60 of children are at risk forheadache in their lifetime with female patients being affectedat a greater rate than male patients21 The prevalence formigraine for those under 20 years of age is 7721 Headachescan become very frequent and evolve into chronic dailyheadache (CDH) a clinical syndrome that continues to beused frequently and defined as ldquopain localized to the headoccurring 15 or more days per month for more than 3monthsrdquo22 Chronic migraine a form of CDH is estimated tooccur in up to 18 of adolescents aged 12ndash17 years23 Whilethere are many pharmacologic treatments available for mi-graine and other primary headache disorders pediatric clinicaltrials including the recent Children and Adolescent MigrainePrevention (CHAMP) study consistently show high placeboresponse rates highlighting the psychological aspects of thedisorder that can be addressed by nonpharmacologic strate-gies Cognitive-behavioral therapy when combined with am-itriptyline has shown to be more effective in reducingheadache frequency and migraine-related disability amongyouth aged 10ndash17 years24 Nonpharmacologic treatment ofheadache such as psychological interventions have beenshown to be as effective as first-line therapy but the frequencyof adoption of this as first-line therapy in clinical practice isunknown25
Cerebral palsyCerebral palsy represents a group of nonprogressive dis-orders due to brain injury that affect postural and motorcontrol resulting in activity limitations26 Estimates from theCDC suggest an incidence of 33 per 1000 live births perCDC data27 Reduction of spasticity and dystonia can resultin increased mobility and decreased pain Medical treat-ments for spasticity and dystonia include oral medicationsor IM injections of botulinum toxin Surgical treatmentsinclude surgical placement of an intrathecal baclofen pumpdeep brain stimulator and selective dorsal rhizotomyMeasures to improve functioning include physical occu-pational and speech therapies as well as the use of orthosesand adaptive equipment28
Global developmental delayGlobal developmental delay (GDD) is diagnosed in childrenwith significant delay in acquiring early childhood de-velopmental milestones in more than 2 domains motorspeech and language cognitive and socialadaptive behav-ior29 The diagnosis is not synonymous with intellectual dis-ability and puts a child at risk for long-term cognitiveimpairments and functional dependence In children withoutautism between 1 and 3 meet criteria for GDD29 Ap-proximately 40 of otherwise unexplained GDD is due togenetic and metabolic disorders rather than environmentalfactors3031 Genetic testing that establishes a specific diagnosishas a number of benefits for patients and families and canresult in important changes in management32
Transition to adult neurologyThe shortage of pediatric neurology providers and increasingnumber of pediatric neurology patients limits the ability of thehealth care system to retain adult patients in the pediatricsystem33 As patients age they have concomitant healthproblems that are more commonly seen in the adult pop-ulation The transition of adolescents with chronic neurologicdisorders from a pediatric to an adult neurologist seems to bea sensible solution though it is a significant challenge3435
Young people referred to adult practitioners from tertiarypediatric centers have more complex health problems Adultproviders report lower levels of confidence in caring for thesepatients36 Other barriers to the transition to adult care are theadolescent and family anxiety of leaving familiar care which isoften family-centered The adult setting is not always equip-ped to handle intellectual disabilities and difficult behaviorwhich is more common in patients with chronic neurologicconditions of pediatric onset33 The American Academy ofPediatrics has outlined a formal transition process to addressthe gaps that currently exist in practice37 A transition plan isessential in ensuring that this occurs
MethodsThe AAN and CNS formed a cross-specialty and multidisci-plinary expert Work Group of diverse key stakeholders fromphysician and nonphysician associations patient and care-giver advocacy organizations and payers Details of the fullmeasure development process are available online38 Theformation of the Work Group began with a nominationprocess from AAN and CNS The co-chairs and facilitatorsselected members from the pool of qualified specialists andexpert nominees The selection was based on the nomineesrsquoexperience in quality measures quality improvement andclinical activities The final Work Group (23) includedphysicians advanced practice providers physical therapistsand a parent advocate (listed at the end of the manuscript)
Initially Work Group members disclosed potential conflictsof interest and completed applications summarizing experi-ences and interests Work Group members were selected
NeurologyorgN Neurology | Volume 90 Number 2 | January 9 2018 3
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
based on experience in various disciplines of child neurologyquality improvement and quality measurement
The measure development process included the following(1) evidence-based literature search (2) establishing a multi-disciplinary Work Group adhering to the AAN conflict ofinterest policy (3) drafting candidate measures and technicalspecifications (4) convening the Work Group in person toreview candidate measures (5) refining and discussing thecandidate measures (6) soliciting public comments on ap-proved measures during a 30-day period (7) refining the finalmeasures according to input received during the publiccomment period and corresponding technical specificationsand (8) obtaining approvals from the Work Group AANQuality and Safety Subcommittee AAN Practice CommitteeAAN Institute Board of Directors and CNS Board ofDirectors
TheWork Group sought to develop evidence-based measuresto support the delivery of high-quality care and to improvepatient outcomes The co-chairs and facilitators guided bya medical librarian conducted a comprehensive literaturesearch identifying 7840 abstracts relevant to the potentialmeasures including 22 clinical practice guidelines Followingthe development of draft measure concepts during the in-personmeeting a public comment period resulted in over 300comments from 60 individuals which drove conceptrefinement
The AAN and CNS plan to provide resources to update thesemeasures every 3 years which provides a working frameworkfor measurement rather than a long-term mandate
Prior to theWork Groupmeeting leadership put forth a set ofcandidate measures for Work Group review and discussionCandidate measures that adapted disease-specific measures tochildren were deferred to future revisions of disease-specificmeasurement sets in order to avoid duplication The WorkGroup met on June 10 2016 reviewed 12 candidate meas-ures and selected and modified 11 of the candidate measuresfor further consideration These measures were further re-fined based on public comments The Work Group approvedthe final 11 measures for inclusion in the AANCNS 2017Child Neurology Quality Measurement Set (table)
ResultsChild Neurology Quality Measurement SetUltimately the Work Group identified 11 quality metrics forimplementation in the care of children with neurologicdisorders
Although rare infantile (epileptic) spasms are a source ofsignificant morbidity mortality and resource expenditure inearly life There is controversy over what antiseizure medi-cation to use first-line but there is good evidence that ACTH
prednisolone or vigabatrin are all reasonable choices39ndash42
Literature suggests that prompt treatment can potentiallylessen the morbidity associated with IS and supports urgencyin initiating appropriate therapy While the precise criticalperiod of the intervention is unknown analysis of time totreatment in one large randomized trial showed that treat-ment initiation beyond 7 days after diagnosis was associatedwith worse developmental outcome at 4 years This suggeststhat initiation of therapy by 7 days may be key to improveddevelopmental outcomes64344
It has been more than a decade since the definition of SE wasnarrowed from a clinical seizure lasting longer than 30 to 5minutes45 Human and animal literature indicates that thesooner prolonged seizures are treated the greater the likeli-hood of termination Thus rescue therapy for children withconvulsive seizures with an appropriate medication at a dosethat is recommended to be effective should be provided forthe prehospital setting The intent is to reduce the morbidityand mortality that can accompany prolonged convulsiveseizures
Refractory SE is currently defined as seizures not responsiveto first- and second-line antiseizure drugs that include onenonbenzodiazepine and typically involves seizures lastinglonger than 30 minutes Although there is a paucity of for-mally conducted trials that assess timing of administration andhead-to-head trials of different agents consensus suggests thatthird-line medications with potential to treat refractory SEshould be administered within 60 minutes of onset in thehospital or arrival in the emergency department16
Children with epilepsy can have a variety of associated neu-rodevelopmental (eg specific learning) and neurobehavioral(eg attention-deficithyperactivity disorder autism spec-trum disorder) disabilities These can adversely affect qualityof life as much as or more than the actual epilepsy syndromeIt has been suggested that rather than comorbidities theseneurodevelopmental and behavioral difficulties are an integralpart of epilepsy Routine use of validated screening tools forneurodevelopmental and neurobehavioral abnormalities canallow for earlier diagnosis and improved outcomes Use ofthese instruments should not add substantially to theresources required for the care of these children with epilepsy
The most common movement disorder seen by child neu-rologists is TD which is seen transiently in childhood andincludes TS Although many tics of childhood are self-limiting common comorbidities include attention-deficithyperactivity disorder oppositional defiant disorderobsessive-compulsive disorder and mood disorders like anx-iety and depression As is the case for the epilepsies thesecomorbid conditions can be extremely debilitating for thechild and family Evaluating for these conditions is possible byasking relatively simple questions in the outpatient settingwith subsequent referral for more precise diagnosis andtreatment as needed The management of tic-related
4 Neurology | Volume 90 Number 2 | January 9 2018 NeurologyorgN
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
comorbidities can be performed by the child neurologist withappropriate pharmacotherapy or by referral to another careprovider like a child psychologist or psychiatrist Frequentlymedications can often be avoided as behavioral therapy forchronic TD and TS can be as effective as pharmacotherapy inreducing the frequency of tics and improving quality of lifeSeveral behavioral therapy approaches can be performed in anoffice setting by a child neurologist or by referral to a providerwho specializes in these treatment paradigms The type oftherapy chosen should be tailored to the needs of the child aswell as her or his ability to cooperate based upon level ofintellectual development and behavioral competence
Despite a variety of pharmacologic options migraine canbecome a chronic and debilitating condition in childhoodPsychological interventions (cognitive-behavioral therapybiofeedback or relaxation therapy) have been demonstratedto provide significant symptom relief and improve quality oflife
Spasticity and dystonia when localized or segmental can havemorbidity including pain and limitation of movement whichcan interfere with hygienecare and with the implementationof other therapies such as orthoses The care of children withspasticity and dystonia is best accomplished by a team ofindividuals to provide multifaceted treatment approachestailored to the needs of the patient These patients should beevaluated (or referred for evaluation) to a care provider ex-perienced in the use of botulinum A toxin which has beendemonstrated to be effective in reducing spasticity and dys-tonia In addition to symptom relief early use of this treat-ment modality can allow for improved function duringa critical period of motor development
GDD is a common problem that is referred to the pediatricneurologist The lack of a definitive diagnosis can delay onsetof therapies for treatable conditions therapy affect discussionrelated to prognosis and genetic counseling and frequentlyleads to unnecessary diagnostic testing The latter can beinvasive (eg lumbar puncture muscle biopsy) expensiveand lead to additional testing that may not be relevant In thiscontext it is reasonable to perform chromosomal microarray(CMA) as first-line testing Although CMA has sensitivity andspecificity limitations (like all other tests) it is the currentgateway to genetic diagnoses of GDD
Preparing patients and families for transition to adult care isone of the most important tasks to be performed in thelongitudinal care of a child with a chronic neurologic dis-order A variety of factors can influence creation and un-derstanding of this care plan including the developmentalstatus of the child as well as the need for and availability ofongoing neurologic care by an adult provider Neverthelessguidelines suggest that a written plan is in the best interest ofthe patient and the written plan should cover the medicalemotional social legal and financial needs associated withtransition to adult life
DiscussionThese quality measures represent a starting point for measuredevelopment in child neurology They are designed to allowchild neurologists to measure performance in areas wherethere are potential gaps in care Further work will be neededto identify and address other existing gaps in care The WorkGroup recognizes that resource gaps exist and may need to beaddressed to ensure success in complying with these qualitymeasures and ongoing advancement of best practices Theproposed quality measures are not intended to be used asproof of failure to provide optimal care when local resourcesmay not be able to accommodate The idea is not for thesemeasures to be used as guidelines or for the purposes ofpunishment for low performance Each child neurology pro-vider should take these measures and evaluate their currentbaseline Then by setting a goal for improvement of carequality improvement methodology can be implemented toimprove the outcomes and lives of patients with pediatricneurologic illnesses
Author contributionsDr Berg and Dr Billinghurst contributed to acquisition ofdata analysis or interpretation of data and critical revisionof the manuscript for important intellectual contentDr Buchhalter contributed to study concept and design ac-quisition of data analysis or interpretation of data draftingrevising the manuscript critical revision of the manuscript forimportant intellectual content and study supervisionDr Fain E Fecske Dr Feyma Dr Grinspan Dr HoutrowDr Kothare and Dr Kumar contributed to acquisition ofdata analysis or interpretation of data and critical revision ofthe manuscript for important intellectual content E Leecontributed to study concept and design acquisition of dataanalysis or interpretation of data draftingrevising the man-uscript critical revision of the manuscript for important in-tellectual content and study supervision Dr Monduy and DrMorita contributed to acquisition of data analysis or in-terpretation of data and critical revision of the manuscript forimportant intellectual content Dr Patel contributed to studyconcept and design acquisition of data analysis or in-terpretation of data draftingrevising the manuscript criticalrevision of the manuscript for important intellectual contentand study supervision Dr Szperka contributed to acquisitionof data analysis or interpretation of data and critical revisionof the manuscript for important intellectual content DrVictorio contributed to study concept and design acquisitionof data analysis or interpretation of data draftingrevising themanuscript critical revision of the manuscript for importantintellectual content and study supervision Dr Yeh contrib-uted to acquisition of data analysis or interpretation of dataand critical revision of the manuscript for important in-tellectual content
AcknowledgmentThe authors thank the Child Neurology Quality Measure-ment Set Work Group members for their dedication time
NeurologyorgN Neurology | Volume 90 Number 2 | January 9 2018 5
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
energy contributions and work that supported the de-velopment of this article Jeffrey Buchhalter MD FAAN(Chair Child Neurology Society) Anup Patel MD FAAN(Chair American Academy of Neurology) Karen Ballaban-Gil MD (American Academy of Neurology) Anne BergPhD (American Academy of Neurology) Daniel Fain MDFAAN (American Academy of Neurology) Cynthia KeatorMD (American Academy of Neurology) Sanjeev KothareMD (American Academy of Neurology) Gogi Kumar MD(American Academy of Neurology) Migvis MonduyMD (American Academy of Neurology) Diego Morita MD(American Academy of Neurology) Zachary Grinspan MDMS (American Academy of Pediatrics Child NeurologySociety) Erin Fecske MSN RN CNRN CPNP (AmericanAssociation of Neuroscience Nurses) Amy Houtrow MDPhD MPH (American Academy of Physical Medicine andRehabilitation) Kevin Chapman MD (American EpilepsySociety) Christina Szperka MD (American Headache Soci-ety) Lynn Jeffries PT DPT PhD PCS (American PhysicalTherapy Association) Tim Feyma MD (Child NeurologySociety) Kiran Maski MD (Child Neurology Society) LisaMeunier (Epilepsy Foundation) Lori Billinghurst MD MScFRCPC (independent) Ann Yeh MD (independent) DavidMichelsonMD (Facilitator AmericanAcademy ofNeurology)M Cristina Victorio MD (Facilitator American Academyof Neurology) Amy Bennett JD (American Academy ofNeurology staff) Gina Gjorvad (American Academy ofNeurology staff) Erin Lee (American Academy of Neurologystaff) Becky Schierman MPH (American Academy ofNeurology staff)
Study fundingNo targeted funding reported
DisclosureA Patel receives research grants from the Pediatric EpilepsyResearch Foundation Upsher-Smith GW Pharmaceuticalsand LivaNova He consults for GW Pharmaceuticals andLivaNova He is on a scientific advisory board for UCBPharma and Supernus A Berg and L Billinghurst report nodisclosures relevant to the manuscript D Fain is a sub-investigator for the following study as of September 6 2016 ARandomized Double-Blind Placebo-Controlled Study toEvaluate the Efficacy and Safety of Sage-547 Injection in theTreatment of Subjects with Super-Refractory Status Epi-lepticus Protocol number 547-SSE-301 E Fecske andT Feyma report no disclosures relevant to the manuscriptZ Grinspan receives funding from the Centers for DiseaseControl and Prevention the Pediatric Epilepsy ResearchFoundation and the Nanette Laitman Clinical Scholars Pro-gram A Houtrow S Kothare and G Kumar report no dis-closures relevant to the manuscript E Lee is an employee ofthe American Academy of Neurology M Monduy andD Morita report no disclosures relevant to the manuscriptC Szperka received funding from Pfizer andMiles for MigraineM Victorio reports no disclosures relevant to themanuscript AYeh receives research funding from the NMSS CMSC IORM
SCN CBMH Chase an Idea SickKids Foundation Rare Dis-eases Foundation MS Scientific Foundation (Canada)MSSociety McLaughlin Centre and Mario Battaglia FoundationShe has received unrestricted funds to run an educationalsymposium from theGuthy Jackson Charitable Foundation andTeva She has served on a scientific advisory board for neuro-toxicity for Juno She serves as a relapse adjudicator for ACI Shehas received honorarium for a talk from Novartis J Buchhalteris on the Scientific Advisory Board of the Charlie FoundationHe receives research funding from the Alberta ChildrenrsquosHospital Research Institute and the Department of PediatricsUniversity of Calgary Alberta Canada Go to NeurologyorgNfor full disclosures
Received May 17 2017 Accepted in final form September 25 2017
References1 Lipton RB Bigal ME The epidemiology of migraine Am J Med 2005118(suppl 1)
3Sndash10S2 Lipton RB Bigal ME Migraine epidemiology impact and risk factors for pro-
gression Headache 200545(suppl 1)S3ndashS133 Knight T Steeves T Day L Lowerison M Jette N Pringsheim T Prevalence of tic
disorders a systematic review and meta-analysis Pediatr Neurol 20124777ndash904 Moreau JF Fink EL Hartman ME et al Hospitalizations of children with neurologic
disorders in the United States Pediatr Crit Care Med 201314801ndash8105 American Academy of Pediatrics Supporting the health care transition from ado-
lescence to adulthood in the medical home Pediatrics 2011128182ndash2006 Kivity S Lerman P Ariel R Danziger Y Mimouni M Shinnar S Long-term cognitive
outcomes of a cohort of children with cryptogenic infantile spasms treated with high-dose adrenocorticotropic hormone Epilepsia 200445255ndash262
7 Eisermann MM DeLaRaillere A Dellatolas G et al Infantile spasms in Down syn-drome effects of delayed anticonvulsive treatment Epilepsy Res 20035521ndash27
8 Goh S Kwiatkowski DJ Dorer DJ Thiele EA Infantile spasms and intellectualoutcomes in children with tuberous sclerosis complex Neurology 200565235ndash238
9 Riikonen R Long-term outcome in children with infantile spasms treated with vig-abatrin a cohort of 180 patients Epilepsia 201556807ndash809
10 Riikonen R A long-term follow-up study of 214 children with the syndrome ofinfantile spasms Neuropediatrics 19821314ndash23
11 Riikonen R Long-term outcome ofWest syndrome a study of adults with a history ofinfantile spasms Epilepsia 199637367ndash372
12 Widjaja E Go C McCoy B Snead O Neurodevelopmental outcome of infantilespasms a systematic review and meta-analysis Epilepsy Res 2015109155ndash162
13 Luthvigsson P Olafsson E Sigurdardottir S Hauser WA Epidemiologic features ofinfantile spasms in Iceland Epilepsia 199435802ndash805
14 Riikonen R Epidemiological data of West syndrome in Finland Brain Dev 200123539ndash541
15 Knupp KG Coryell J Nickels KC et al Response to treatment in a prospectivenational infantile spasms cohort Ann Neurol 201679475ndash484
16 Glauser T Shinnar S Gloss D et al Evidence-based guideline treatment of con-vulsive status epilepticus in children and adults report of the guideline committee ofthe American Epilepsy Society Epilepsy Curr 20161648ndash61
17 Brophy GM Bell R Claassen J et al Guidelines for the evaluation andmanagement ofstatus epilepticus Neurocrit Care 2012173ndash23
18 Cath D Hedderly T Ludolph A et al European clinical guidelines for Tourettesyndrome and other tic disorders part I assessment Eur Child Adolesc Psychiatry201120155ndash171
19 Knight T Steeves T Day L et al Prevalence of tic disorders a systematic review andmeta-analysis Pediatr Neurol 20124777ndash90
20 Tourette Syndrome Fact Sheet NIH Available at nindsnihgovdisorderstourettedetail_tourettehtm Accessed April 20 2016
21 Abu-Arafeh I Razak S Sivaraman B Graham C Prevalence of headache and migrainein children and adolescents a systematic review of population-based studies DevMedChild Neurol 2010521088ndash1097
22 Chiappedi M Mensi MM Termine C Balottin U Psychological therapy in adoles-cents with chronic daily headache Curr Pain Headache Rep 2016203
23 Lipton RB Nanack A Ricci JA et al Prevalence and burden of chronic migraine inadolescents results of the chronic daily headache in adolescents study (C-dAS)Headache 201151693ndash706
24 Powers SW Kashikar-Zuck SM Allen JR et al Cognitive behavioral therapy plusamitriptyline for chronic migraine in children and adolescents a randomized clinicaltrial JAMA 20133102622ndash2630
25 Faedda N Cerutti R Verdecchia P et al Behavioral management of headache inchildren and adolescents J Headache Pain 20161780
26 Bax M Goldstein M Rosenbaum P et al Executive Committee for the Definition ofCerebral Palsy Proposed definition and classification of cerebral palsy April 2005Dev Med Child Neurol 200547571ndash576
6 Neurology | Volume 90 Number 2 | January 9 2018 NeurologyorgN
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
27 Cerebral Palsy Fact Sheet NIH Available at nindsnihgovdisorderscerebral_palsydetail_cerebral_palsyhtm3104_2 Accessed September 3 2017
28 Shamsoddini A Amirsalari S Hollisaz MT Rahimnia A Khatibi-Aghda A Man-agement of spasticity in children with cerebral palsy Iranian J Pediatr 201424345ndash351
29 Michelson D Shevell M Sheer E et al Evidence report genetic and metabolic testingon children with global developmental delay Neurology 2011771629ndash1635
30 Kaufman L Ayub M Vincent JB The genetic basis of non-syndromic intellectualdisability a review J Neurodev Disord 20102182ndash209
31 Rauch A Hoyer J Guth S et al Diagnostic yield of various genetic approaches inpatients with unexplained developmental delay or mental retardation Am J MedGenet A 20061402063ndash2074
32 Riggs E Wain K Riethmaier D et al Chromosomal microarray impacts clinicalmanagement Clin Genet 201385147ndash153
33 Camfield P Gibson P Douglass L Strategies for transitioning to adult care for youthwith Lennox-Gastaut syndrome and related disorders Epilepsia 20115221ndash27
34 Brown L Camfield P Capers M et al The neurologistrsquos role in supporting transitionto adult health care Neurology 2016871ndash6
35 Camfield P Camfield C Transition to adult care for children with chronic neuro-logical disorders Ann Neurol 201169437ndash444
36 Borlot F Tellez-Zenteno JF Allen A Ali A Snead OC III Andrade DM Epilepsytransition challenges of caring for adults with childhood-onset seizures Epilepsia2014551659ndash1666
37 American Academy of Pediatrics Clinical report supporting the health care transitionfrom adolescence to adulthood in the medical home Pediatrics 2011128182ndash200
38 American Academy of Neurology Quality measurement manual 2014 Update Availableat aancomuploadedFilesWebsite_Library_AssetsDocuments3Practice_Manage-ment2Quality_Improvement1Quality_Measures2About_Quality_Measures20152002201120Process20Manual20Finalpdf Accessed March 9 2017
39 Go C Mackay M Weiss S et al Evidence-based guideline update medical treatmentof infantile spasms Neurology 2012781974ndash1980
40 Jones K Boyd J Go C et al Vigabatrin in the first line treatment of infantile spasmsEpilepsy Currents 201515533ndash534
41 Jones K Go C ACTH vs prednisolone in the treatment of infantile spasms postvigabatrin failure Epilepsy Currents 201414447ndash448
42 Jones K Go C Boyd J et al Vigabatrin as first-line treatment for infantile spasms notrelated to tuberous sclerosis complex Pediatr Neurol 201553141ndash145
43 Chapman KE Specchio N Shinnar S Holmes GL Seizing control of epileptic activitycan improve outcome Epilepsia 2015561482ndash1485
44 OrsquoCallaghan FJ Lux AL Darke K et al The effect of lead time to treatment andof age of onset on developmental outcome at 4 years in infantile spasms evi-dence from the United Kingdom Infantile Spasms Study Epilepsia 2011521359ndash1364
45 Trinka E Cock H Hesdorffer D et al A definition and classification of status epi-lepticus report of the ILAE Task Force on Classification of Status Epilepticus Epi-lepsia 2015561515ndash1523
NeurologyorgN Neurology | Volume 90 Number 2 | January 9 2018 7
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
DOI 101212WNL0000000000004806 published online December 15 2017Neurology
Anup D Patel Anne T Berg Lori Billinghurst et al summary
Quality improvement in neurology Child neurology quality measure set Executive
This information is current as of December 15 2017
ServicesUpdated Information amp
806fullhtmlhttpnneurologyorgcontentearly20171215WNL0000000000004including high resolution figures can be found at
Citations
806fullhtmlotherarticleshttpnneurologyorgcontentearly20171215WNL0000000000004This article has been cited by 1 HighWire-hosted articles
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httpnneurologyorgcgicollectionall_pediatricAll Pediatric
httpnneurologyorgcgicollectionall_health_services_researchAll Health Services Researchfollowing collection(s) This article along with others on similar topics appears in the
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httpnneurologyorgmiscaboutxhtmlpermissionsits entirety can be found online atInformation about reproducing this article in parts (figurestables) or in
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Neurology All rights reserved Print ISSN 0028-3878 Online ISSN 1526-632X1951 it is now a weekly with 48 issues per year Copyright Copyright copy 2017 American Academy of
reg is the official journal of the American Academy of Neurology Published continuously sinceNeurology
based on experience in various disciplines of child neurologyquality improvement and quality measurement
The measure development process included the following(1) evidence-based literature search (2) establishing a multi-disciplinary Work Group adhering to the AAN conflict ofinterest policy (3) drafting candidate measures and technicalspecifications (4) convening the Work Group in person toreview candidate measures (5) refining and discussing thecandidate measures (6) soliciting public comments on ap-proved measures during a 30-day period (7) refining the finalmeasures according to input received during the publiccomment period and corresponding technical specificationsand (8) obtaining approvals from the Work Group AANQuality and Safety Subcommittee AAN Practice CommitteeAAN Institute Board of Directors and CNS Board ofDirectors
TheWork Group sought to develop evidence-based measuresto support the delivery of high-quality care and to improvepatient outcomes The co-chairs and facilitators guided bya medical librarian conducted a comprehensive literaturesearch identifying 7840 abstracts relevant to the potentialmeasures including 22 clinical practice guidelines Followingthe development of draft measure concepts during the in-personmeeting a public comment period resulted in over 300comments from 60 individuals which drove conceptrefinement
The AAN and CNS plan to provide resources to update thesemeasures every 3 years which provides a working frameworkfor measurement rather than a long-term mandate
Prior to theWork Groupmeeting leadership put forth a set ofcandidate measures for Work Group review and discussionCandidate measures that adapted disease-specific measures tochildren were deferred to future revisions of disease-specificmeasurement sets in order to avoid duplication The WorkGroup met on June 10 2016 reviewed 12 candidate meas-ures and selected and modified 11 of the candidate measuresfor further consideration These measures were further re-fined based on public comments The Work Group approvedthe final 11 measures for inclusion in the AANCNS 2017Child Neurology Quality Measurement Set (table)
ResultsChild Neurology Quality Measurement SetUltimately the Work Group identified 11 quality metrics forimplementation in the care of children with neurologicdisorders
Although rare infantile (epileptic) spasms are a source ofsignificant morbidity mortality and resource expenditure inearly life There is controversy over what antiseizure medi-cation to use first-line but there is good evidence that ACTH
prednisolone or vigabatrin are all reasonable choices39ndash42
Literature suggests that prompt treatment can potentiallylessen the morbidity associated with IS and supports urgencyin initiating appropriate therapy While the precise criticalperiod of the intervention is unknown analysis of time totreatment in one large randomized trial showed that treat-ment initiation beyond 7 days after diagnosis was associatedwith worse developmental outcome at 4 years This suggeststhat initiation of therapy by 7 days may be key to improveddevelopmental outcomes64344
It has been more than a decade since the definition of SE wasnarrowed from a clinical seizure lasting longer than 30 to 5minutes45 Human and animal literature indicates that thesooner prolonged seizures are treated the greater the likeli-hood of termination Thus rescue therapy for children withconvulsive seizures with an appropriate medication at a dosethat is recommended to be effective should be provided forthe prehospital setting The intent is to reduce the morbidityand mortality that can accompany prolonged convulsiveseizures
Refractory SE is currently defined as seizures not responsiveto first- and second-line antiseizure drugs that include onenonbenzodiazepine and typically involves seizures lastinglonger than 30 minutes Although there is a paucity of for-mally conducted trials that assess timing of administration andhead-to-head trials of different agents consensus suggests thatthird-line medications with potential to treat refractory SEshould be administered within 60 minutes of onset in thehospital or arrival in the emergency department16
Children with epilepsy can have a variety of associated neu-rodevelopmental (eg specific learning) and neurobehavioral(eg attention-deficithyperactivity disorder autism spec-trum disorder) disabilities These can adversely affect qualityof life as much as or more than the actual epilepsy syndromeIt has been suggested that rather than comorbidities theseneurodevelopmental and behavioral difficulties are an integralpart of epilepsy Routine use of validated screening tools forneurodevelopmental and neurobehavioral abnormalities canallow for earlier diagnosis and improved outcomes Use ofthese instruments should not add substantially to theresources required for the care of these children with epilepsy
The most common movement disorder seen by child neu-rologists is TD which is seen transiently in childhood andincludes TS Although many tics of childhood are self-limiting common comorbidities include attention-deficithyperactivity disorder oppositional defiant disorderobsessive-compulsive disorder and mood disorders like anx-iety and depression As is the case for the epilepsies thesecomorbid conditions can be extremely debilitating for thechild and family Evaluating for these conditions is possible byasking relatively simple questions in the outpatient settingwith subsequent referral for more precise diagnosis andtreatment as needed The management of tic-related
4 Neurology | Volume 90 Number 2 | January 9 2018 NeurologyorgN
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
comorbidities can be performed by the child neurologist withappropriate pharmacotherapy or by referral to another careprovider like a child psychologist or psychiatrist Frequentlymedications can often be avoided as behavioral therapy forchronic TD and TS can be as effective as pharmacotherapy inreducing the frequency of tics and improving quality of lifeSeveral behavioral therapy approaches can be performed in anoffice setting by a child neurologist or by referral to a providerwho specializes in these treatment paradigms The type oftherapy chosen should be tailored to the needs of the child aswell as her or his ability to cooperate based upon level ofintellectual development and behavioral competence
Despite a variety of pharmacologic options migraine canbecome a chronic and debilitating condition in childhoodPsychological interventions (cognitive-behavioral therapybiofeedback or relaxation therapy) have been demonstratedto provide significant symptom relief and improve quality oflife
Spasticity and dystonia when localized or segmental can havemorbidity including pain and limitation of movement whichcan interfere with hygienecare and with the implementationof other therapies such as orthoses The care of children withspasticity and dystonia is best accomplished by a team ofindividuals to provide multifaceted treatment approachestailored to the needs of the patient These patients should beevaluated (or referred for evaluation) to a care provider ex-perienced in the use of botulinum A toxin which has beendemonstrated to be effective in reducing spasticity and dys-tonia In addition to symptom relief early use of this treat-ment modality can allow for improved function duringa critical period of motor development
GDD is a common problem that is referred to the pediatricneurologist The lack of a definitive diagnosis can delay onsetof therapies for treatable conditions therapy affect discussionrelated to prognosis and genetic counseling and frequentlyleads to unnecessary diagnostic testing The latter can beinvasive (eg lumbar puncture muscle biopsy) expensiveand lead to additional testing that may not be relevant In thiscontext it is reasonable to perform chromosomal microarray(CMA) as first-line testing Although CMA has sensitivity andspecificity limitations (like all other tests) it is the currentgateway to genetic diagnoses of GDD
Preparing patients and families for transition to adult care isone of the most important tasks to be performed in thelongitudinal care of a child with a chronic neurologic dis-order A variety of factors can influence creation and un-derstanding of this care plan including the developmentalstatus of the child as well as the need for and availability ofongoing neurologic care by an adult provider Neverthelessguidelines suggest that a written plan is in the best interest ofthe patient and the written plan should cover the medicalemotional social legal and financial needs associated withtransition to adult life
DiscussionThese quality measures represent a starting point for measuredevelopment in child neurology They are designed to allowchild neurologists to measure performance in areas wherethere are potential gaps in care Further work will be neededto identify and address other existing gaps in care The WorkGroup recognizes that resource gaps exist and may need to beaddressed to ensure success in complying with these qualitymeasures and ongoing advancement of best practices Theproposed quality measures are not intended to be used asproof of failure to provide optimal care when local resourcesmay not be able to accommodate The idea is not for thesemeasures to be used as guidelines or for the purposes ofpunishment for low performance Each child neurology pro-vider should take these measures and evaluate their currentbaseline Then by setting a goal for improvement of carequality improvement methodology can be implemented toimprove the outcomes and lives of patients with pediatricneurologic illnesses
Author contributionsDr Berg and Dr Billinghurst contributed to acquisition ofdata analysis or interpretation of data and critical revisionof the manuscript for important intellectual contentDr Buchhalter contributed to study concept and design ac-quisition of data analysis or interpretation of data draftingrevising the manuscript critical revision of the manuscript forimportant intellectual content and study supervisionDr Fain E Fecske Dr Feyma Dr Grinspan Dr HoutrowDr Kothare and Dr Kumar contributed to acquisition ofdata analysis or interpretation of data and critical revision ofthe manuscript for important intellectual content E Leecontributed to study concept and design acquisition of dataanalysis or interpretation of data draftingrevising the man-uscript critical revision of the manuscript for important in-tellectual content and study supervision Dr Monduy and DrMorita contributed to acquisition of data analysis or in-terpretation of data and critical revision of the manuscript forimportant intellectual content Dr Patel contributed to studyconcept and design acquisition of data analysis or in-terpretation of data draftingrevising the manuscript criticalrevision of the manuscript for important intellectual contentand study supervision Dr Szperka contributed to acquisitionof data analysis or interpretation of data and critical revisionof the manuscript for important intellectual content DrVictorio contributed to study concept and design acquisitionof data analysis or interpretation of data draftingrevising themanuscript critical revision of the manuscript for importantintellectual content and study supervision Dr Yeh contrib-uted to acquisition of data analysis or interpretation of dataand critical revision of the manuscript for important in-tellectual content
AcknowledgmentThe authors thank the Child Neurology Quality Measure-ment Set Work Group members for their dedication time
NeurologyorgN Neurology | Volume 90 Number 2 | January 9 2018 5
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
energy contributions and work that supported the de-velopment of this article Jeffrey Buchhalter MD FAAN(Chair Child Neurology Society) Anup Patel MD FAAN(Chair American Academy of Neurology) Karen Ballaban-Gil MD (American Academy of Neurology) Anne BergPhD (American Academy of Neurology) Daniel Fain MDFAAN (American Academy of Neurology) Cynthia KeatorMD (American Academy of Neurology) Sanjeev KothareMD (American Academy of Neurology) Gogi Kumar MD(American Academy of Neurology) Migvis MonduyMD (American Academy of Neurology) Diego Morita MD(American Academy of Neurology) Zachary Grinspan MDMS (American Academy of Pediatrics Child NeurologySociety) Erin Fecske MSN RN CNRN CPNP (AmericanAssociation of Neuroscience Nurses) Amy Houtrow MDPhD MPH (American Academy of Physical Medicine andRehabilitation) Kevin Chapman MD (American EpilepsySociety) Christina Szperka MD (American Headache Soci-ety) Lynn Jeffries PT DPT PhD PCS (American PhysicalTherapy Association) Tim Feyma MD (Child NeurologySociety) Kiran Maski MD (Child Neurology Society) LisaMeunier (Epilepsy Foundation) Lori Billinghurst MD MScFRCPC (independent) Ann Yeh MD (independent) DavidMichelsonMD (Facilitator AmericanAcademy ofNeurology)M Cristina Victorio MD (Facilitator American Academyof Neurology) Amy Bennett JD (American Academy ofNeurology staff) Gina Gjorvad (American Academy ofNeurology staff) Erin Lee (American Academy of Neurologystaff) Becky Schierman MPH (American Academy ofNeurology staff)
Study fundingNo targeted funding reported
DisclosureA Patel receives research grants from the Pediatric EpilepsyResearch Foundation Upsher-Smith GW Pharmaceuticalsand LivaNova He consults for GW Pharmaceuticals andLivaNova He is on a scientific advisory board for UCBPharma and Supernus A Berg and L Billinghurst report nodisclosures relevant to the manuscript D Fain is a sub-investigator for the following study as of September 6 2016 ARandomized Double-Blind Placebo-Controlled Study toEvaluate the Efficacy and Safety of Sage-547 Injection in theTreatment of Subjects with Super-Refractory Status Epi-lepticus Protocol number 547-SSE-301 E Fecske andT Feyma report no disclosures relevant to the manuscriptZ Grinspan receives funding from the Centers for DiseaseControl and Prevention the Pediatric Epilepsy ResearchFoundation and the Nanette Laitman Clinical Scholars Pro-gram A Houtrow S Kothare and G Kumar report no dis-closures relevant to the manuscript E Lee is an employee ofthe American Academy of Neurology M Monduy andD Morita report no disclosures relevant to the manuscriptC Szperka received funding from Pfizer andMiles for MigraineM Victorio reports no disclosures relevant to themanuscript AYeh receives research funding from the NMSS CMSC IORM
SCN CBMH Chase an Idea SickKids Foundation Rare Dis-eases Foundation MS Scientific Foundation (Canada)MSSociety McLaughlin Centre and Mario Battaglia FoundationShe has received unrestricted funds to run an educationalsymposium from theGuthy Jackson Charitable Foundation andTeva She has served on a scientific advisory board for neuro-toxicity for Juno She serves as a relapse adjudicator for ACI Shehas received honorarium for a talk from Novartis J Buchhalteris on the Scientific Advisory Board of the Charlie FoundationHe receives research funding from the Alberta ChildrenrsquosHospital Research Institute and the Department of PediatricsUniversity of Calgary Alberta Canada Go to NeurologyorgNfor full disclosures
Received May 17 2017 Accepted in final form September 25 2017
References1 Lipton RB Bigal ME The epidemiology of migraine Am J Med 2005118(suppl 1)
3Sndash10S2 Lipton RB Bigal ME Migraine epidemiology impact and risk factors for pro-
gression Headache 200545(suppl 1)S3ndashS133 Knight T Steeves T Day L Lowerison M Jette N Pringsheim T Prevalence of tic
disorders a systematic review and meta-analysis Pediatr Neurol 20124777ndash904 Moreau JF Fink EL Hartman ME et al Hospitalizations of children with neurologic
disorders in the United States Pediatr Crit Care Med 201314801ndash8105 American Academy of Pediatrics Supporting the health care transition from ado-
lescence to adulthood in the medical home Pediatrics 2011128182ndash2006 Kivity S Lerman P Ariel R Danziger Y Mimouni M Shinnar S Long-term cognitive
outcomes of a cohort of children with cryptogenic infantile spasms treated with high-dose adrenocorticotropic hormone Epilepsia 200445255ndash262
7 Eisermann MM DeLaRaillere A Dellatolas G et al Infantile spasms in Down syn-drome effects of delayed anticonvulsive treatment Epilepsy Res 20035521ndash27
8 Goh S Kwiatkowski DJ Dorer DJ Thiele EA Infantile spasms and intellectualoutcomes in children with tuberous sclerosis complex Neurology 200565235ndash238
9 Riikonen R Long-term outcome in children with infantile spasms treated with vig-abatrin a cohort of 180 patients Epilepsia 201556807ndash809
10 Riikonen R A long-term follow-up study of 214 children with the syndrome ofinfantile spasms Neuropediatrics 19821314ndash23
11 Riikonen R Long-term outcome ofWest syndrome a study of adults with a history ofinfantile spasms Epilepsia 199637367ndash372
12 Widjaja E Go C McCoy B Snead O Neurodevelopmental outcome of infantilespasms a systematic review and meta-analysis Epilepsy Res 2015109155ndash162
13 Luthvigsson P Olafsson E Sigurdardottir S Hauser WA Epidemiologic features ofinfantile spasms in Iceland Epilepsia 199435802ndash805
14 Riikonen R Epidemiological data of West syndrome in Finland Brain Dev 200123539ndash541
15 Knupp KG Coryell J Nickels KC et al Response to treatment in a prospectivenational infantile spasms cohort Ann Neurol 201679475ndash484
16 Glauser T Shinnar S Gloss D et al Evidence-based guideline treatment of con-vulsive status epilepticus in children and adults report of the guideline committee ofthe American Epilepsy Society Epilepsy Curr 20161648ndash61
17 Brophy GM Bell R Claassen J et al Guidelines for the evaluation andmanagement ofstatus epilepticus Neurocrit Care 2012173ndash23
18 Cath D Hedderly T Ludolph A et al European clinical guidelines for Tourettesyndrome and other tic disorders part I assessment Eur Child Adolesc Psychiatry201120155ndash171
19 Knight T Steeves T Day L et al Prevalence of tic disorders a systematic review andmeta-analysis Pediatr Neurol 20124777ndash90
20 Tourette Syndrome Fact Sheet NIH Available at nindsnihgovdisorderstourettedetail_tourettehtm Accessed April 20 2016
21 Abu-Arafeh I Razak S Sivaraman B Graham C Prevalence of headache and migrainein children and adolescents a systematic review of population-based studies DevMedChild Neurol 2010521088ndash1097
22 Chiappedi M Mensi MM Termine C Balottin U Psychological therapy in adoles-cents with chronic daily headache Curr Pain Headache Rep 2016203
23 Lipton RB Nanack A Ricci JA et al Prevalence and burden of chronic migraine inadolescents results of the chronic daily headache in adolescents study (C-dAS)Headache 201151693ndash706
24 Powers SW Kashikar-Zuck SM Allen JR et al Cognitive behavioral therapy plusamitriptyline for chronic migraine in children and adolescents a randomized clinicaltrial JAMA 20133102622ndash2630
25 Faedda N Cerutti R Verdecchia P et al Behavioral management of headache inchildren and adolescents J Headache Pain 20161780
26 Bax M Goldstein M Rosenbaum P et al Executive Committee for the Definition ofCerebral Palsy Proposed definition and classification of cerebral palsy April 2005Dev Med Child Neurol 200547571ndash576
6 Neurology | Volume 90 Number 2 | January 9 2018 NeurologyorgN
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
27 Cerebral Palsy Fact Sheet NIH Available at nindsnihgovdisorderscerebral_palsydetail_cerebral_palsyhtm3104_2 Accessed September 3 2017
28 Shamsoddini A Amirsalari S Hollisaz MT Rahimnia A Khatibi-Aghda A Man-agement of spasticity in children with cerebral palsy Iranian J Pediatr 201424345ndash351
29 Michelson D Shevell M Sheer E et al Evidence report genetic and metabolic testingon children with global developmental delay Neurology 2011771629ndash1635
30 Kaufman L Ayub M Vincent JB The genetic basis of non-syndromic intellectualdisability a review J Neurodev Disord 20102182ndash209
31 Rauch A Hoyer J Guth S et al Diagnostic yield of various genetic approaches inpatients with unexplained developmental delay or mental retardation Am J MedGenet A 20061402063ndash2074
32 Riggs E Wain K Riethmaier D et al Chromosomal microarray impacts clinicalmanagement Clin Genet 201385147ndash153
33 Camfield P Gibson P Douglass L Strategies for transitioning to adult care for youthwith Lennox-Gastaut syndrome and related disorders Epilepsia 20115221ndash27
34 Brown L Camfield P Capers M et al The neurologistrsquos role in supporting transitionto adult health care Neurology 2016871ndash6
35 Camfield P Camfield C Transition to adult care for children with chronic neuro-logical disorders Ann Neurol 201169437ndash444
36 Borlot F Tellez-Zenteno JF Allen A Ali A Snead OC III Andrade DM Epilepsytransition challenges of caring for adults with childhood-onset seizures Epilepsia2014551659ndash1666
37 American Academy of Pediatrics Clinical report supporting the health care transitionfrom adolescence to adulthood in the medical home Pediatrics 2011128182ndash200
38 American Academy of Neurology Quality measurement manual 2014 Update Availableat aancomuploadedFilesWebsite_Library_AssetsDocuments3Practice_Manage-ment2Quality_Improvement1Quality_Measures2About_Quality_Measures20152002201120Process20Manual20Finalpdf Accessed March 9 2017
39 Go C Mackay M Weiss S et al Evidence-based guideline update medical treatmentof infantile spasms Neurology 2012781974ndash1980
40 Jones K Boyd J Go C et al Vigabatrin in the first line treatment of infantile spasmsEpilepsy Currents 201515533ndash534
41 Jones K Go C ACTH vs prednisolone in the treatment of infantile spasms postvigabatrin failure Epilepsy Currents 201414447ndash448
42 Jones K Go C Boyd J et al Vigabatrin as first-line treatment for infantile spasms notrelated to tuberous sclerosis complex Pediatr Neurol 201553141ndash145
43 Chapman KE Specchio N Shinnar S Holmes GL Seizing control of epileptic activitycan improve outcome Epilepsia 2015561482ndash1485
44 OrsquoCallaghan FJ Lux AL Darke K et al The effect of lead time to treatment andof age of onset on developmental outcome at 4 years in infantile spasms evi-dence from the United Kingdom Infantile Spasms Study Epilepsia 2011521359ndash1364
45 Trinka E Cock H Hesdorffer D et al A definition and classification of status epi-lepticus report of the ILAE Task Force on Classification of Status Epilepticus Epi-lepsia 2015561515ndash1523
NeurologyorgN Neurology | Volume 90 Number 2 | January 9 2018 7
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
DOI 101212WNL0000000000004806 published online December 15 2017Neurology
Anup D Patel Anne T Berg Lori Billinghurst et al summary
Quality improvement in neurology Child neurology quality measure set Executive
This information is current as of December 15 2017
ServicesUpdated Information amp
806fullhtmlhttpnneurologyorgcontentearly20171215WNL0000000000004including high resolution figures can be found at
Citations
806fullhtmlotherarticleshttpnneurologyorgcontentearly20171215WNL0000000000004This article has been cited by 1 HighWire-hosted articles
Subspecialty Collections
httpnneurologyorgcgicollectionall_pediatricAll Pediatric
httpnneurologyorgcgicollectionall_health_services_researchAll Health Services Researchfollowing collection(s) This article along with others on similar topics appears in the
Permissions amp Licensing
httpnneurologyorgmiscaboutxhtmlpermissionsits entirety can be found online atInformation about reproducing this article in parts (figurestables) or in
Reprints
httpnneurologyorgmiscaddirxhtmlreprintsusInformation about ordering reprints can be found online
Neurology All rights reserved Print ISSN 0028-3878 Online ISSN 1526-632X1951 it is now a weekly with 48 issues per year Copyright Copyright copy 2017 American Academy of
reg is the official journal of the American Academy of Neurology Published continuously sinceNeurology
comorbidities can be performed by the child neurologist withappropriate pharmacotherapy or by referral to another careprovider like a child psychologist or psychiatrist Frequentlymedications can often be avoided as behavioral therapy forchronic TD and TS can be as effective as pharmacotherapy inreducing the frequency of tics and improving quality of lifeSeveral behavioral therapy approaches can be performed in anoffice setting by a child neurologist or by referral to a providerwho specializes in these treatment paradigms The type oftherapy chosen should be tailored to the needs of the child aswell as her or his ability to cooperate based upon level ofintellectual development and behavioral competence
Despite a variety of pharmacologic options migraine canbecome a chronic and debilitating condition in childhoodPsychological interventions (cognitive-behavioral therapybiofeedback or relaxation therapy) have been demonstratedto provide significant symptom relief and improve quality oflife
Spasticity and dystonia when localized or segmental can havemorbidity including pain and limitation of movement whichcan interfere with hygienecare and with the implementationof other therapies such as orthoses The care of children withspasticity and dystonia is best accomplished by a team ofindividuals to provide multifaceted treatment approachestailored to the needs of the patient These patients should beevaluated (or referred for evaluation) to a care provider ex-perienced in the use of botulinum A toxin which has beendemonstrated to be effective in reducing spasticity and dys-tonia In addition to symptom relief early use of this treat-ment modality can allow for improved function duringa critical period of motor development
GDD is a common problem that is referred to the pediatricneurologist The lack of a definitive diagnosis can delay onsetof therapies for treatable conditions therapy affect discussionrelated to prognosis and genetic counseling and frequentlyleads to unnecessary diagnostic testing The latter can beinvasive (eg lumbar puncture muscle biopsy) expensiveand lead to additional testing that may not be relevant In thiscontext it is reasonable to perform chromosomal microarray(CMA) as first-line testing Although CMA has sensitivity andspecificity limitations (like all other tests) it is the currentgateway to genetic diagnoses of GDD
Preparing patients and families for transition to adult care isone of the most important tasks to be performed in thelongitudinal care of a child with a chronic neurologic dis-order A variety of factors can influence creation and un-derstanding of this care plan including the developmentalstatus of the child as well as the need for and availability ofongoing neurologic care by an adult provider Neverthelessguidelines suggest that a written plan is in the best interest ofthe patient and the written plan should cover the medicalemotional social legal and financial needs associated withtransition to adult life
DiscussionThese quality measures represent a starting point for measuredevelopment in child neurology They are designed to allowchild neurologists to measure performance in areas wherethere are potential gaps in care Further work will be neededto identify and address other existing gaps in care The WorkGroup recognizes that resource gaps exist and may need to beaddressed to ensure success in complying with these qualitymeasures and ongoing advancement of best practices Theproposed quality measures are not intended to be used asproof of failure to provide optimal care when local resourcesmay not be able to accommodate The idea is not for thesemeasures to be used as guidelines or for the purposes ofpunishment for low performance Each child neurology pro-vider should take these measures and evaluate their currentbaseline Then by setting a goal for improvement of carequality improvement methodology can be implemented toimprove the outcomes and lives of patients with pediatricneurologic illnesses
Author contributionsDr Berg and Dr Billinghurst contributed to acquisition ofdata analysis or interpretation of data and critical revisionof the manuscript for important intellectual contentDr Buchhalter contributed to study concept and design ac-quisition of data analysis or interpretation of data draftingrevising the manuscript critical revision of the manuscript forimportant intellectual content and study supervisionDr Fain E Fecske Dr Feyma Dr Grinspan Dr HoutrowDr Kothare and Dr Kumar contributed to acquisition ofdata analysis or interpretation of data and critical revision ofthe manuscript for important intellectual content E Leecontributed to study concept and design acquisition of dataanalysis or interpretation of data draftingrevising the man-uscript critical revision of the manuscript for important in-tellectual content and study supervision Dr Monduy and DrMorita contributed to acquisition of data analysis or in-terpretation of data and critical revision of the manuscript forimportant intellectual content Dr Patel contributed to studyconcept and design acquisition of data analysis or in-terpretation of data draftingrevising the manuscript criticalrevision of the manuscript for important intellectual contentand study supervision Dr Szperka contributed to acquisitionof data analysis or interpretation of data and critical revisionof the manuscript for important intellectual content DrVictorio contributed to study concept and design acquisitionof data analysis or interpretation of data draftingrevising themanuscript critical revision of the manuscript for importantintellectual content and study supervision Dr Yeh contrib-uted to acquisition of data analysis or interpretation of dataand critical revision of the manuscript for important in-tellectual content
AcknowledgmentThe authors thank the Child Neurology Quality Measure-ment Set Work Group members for their dedication time
NeurologyorgN Neurology | Volume 90 Number 2 | January 9 2018 5
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
energy contributions and work that supported the de-velopment of this article Jeffrey Buchhalter MD FAAN(Chair Child Neurology Society) Anup Patel MD FAAN(Chair American Academy of Neurology) Karen Ballaban-Gil MD (American Academy of Neurology) Anne BergPhD (American Academy of Neurology) Daniel Fain MDFAAN (American Academy of Neurology) Cynthia KeatorMD (American Academy of Neurology) Sanjeev KothareMD (American Academy of Neurology) Gogi Kumar MD(American Academy of Neurology) Migvis MonduyMD (American Academy of Neurology) Diego Morita MD(American Academy of Neurology) Zachary Grinspan MDMS (American Academy of Pediatrics Child NeurologySociety) Erin Fecske MSN RN CNRN CPNP (AmericanAssociation of Neuroscience Nurses) Amy Houtrow MDPhD MPH (American Academy of Physical Medicine andRehabilitation) Kevin Chapman MD (American EpilepsySociety) Christina Szperka MD (American Headache Soci-ety) Lynn Jeffries PT DPT PhD PCS (American PhysicalTherapy Association) Tim Feyma MD (Child NeurologySociety) Kiran Maski MD (Child Neurology Society) LisaMeunier (Epilepsy Foundation) Lori Billinghurst MD MScFRCPC (independent) Ann Yeh MD (independent) DavidMichelsonMD (Facilitator AmericanAcademy ofNeurology)M Cristina Victorio MD (Facilitator American Academyof Neurology) Amy Bennett JD (American Academy ofNeurology staff) Gina Gjorvad (American Academy ofNeurology staff) Erin Lee (American Academy of Neurologystaff) Becky Schierman MPH (American Academy ofNeurology staff)
Study fundingNo targeted funding reported
DisclosureA Patel receives research grants from the Pediatric EpilepsyResearch Foundation Upsher-Smith GW Pharmaceuticalsand LivaNova He consults for GW Pharmaceuticals andLivaNova He is on a scientific advisory board for UCBPharma and Supernus A Berg and L Billinghurst report nodisclosures relevant to the manuscript D Fain is a sub-investigator for the following study as of September 6 2016 ARandomized Double-Blind Placebo-Controlled Study toEvaluate the Efficacy and Safety of Sage-547 Injection in theTreatment of Subjects with Super-Refractory Status Epi-lepticus Protocol number 547-SSE-301 E Fecske andT Feyma report no disclosures relevant to the manuscriptZ Grinspan receives funding from the Centers for DiseaseControl and Prevention the Pediatric Epilepsy ResearchFoundation and the Nanette Laitman Clinical Scholars Pro-gram A Houtrow S Kothare and G Kumar report no dis-closures relevant to the manuscript E Lee is an employee ofthe American Academy of Neurology M Monduy andD Morita report no disclosures relevant to the manuscriptC Szperka received funding from Pfizer andMiles for MigraineM Victorio reports no disclosures relevant to themanuscript AYeh receives research funding from the NMSS CMSC IORM
SCN CBMH Chase an Idea SickKids Foundation Rare Dis-eases Foundation MS Scientific Foundation (Canada)MSSociety McLaughlin Centre and Mario Battaglia FoundationShe has received unrestricted funds to run an educationalsymposium from theGuthy Jackson Charitable Foundation andTeva She has served on a scientific advisory board for neuro-toxicity for Juno She serves as a relapse adjudicator for ACI Shehas received honorarium for a talk from Novartis J Buchhalteris on the Scientific Advisory Board of the Charlie FoundationHe receives research funding from the Alberta ChildrenrsquosHospital Research Institute and the Department of PediatricsUniversity of Calgary Alberta Canada Go to NeurologyorgNfor full disclosures
Received May 17 2017 Accepted in final form September 25 2017
References1 Lipton RB Bigal ME The epidemiology of migraine Am J Med 2005118(suppl 1)
3Sndash10S2 Lipton RB Bigal ME Migraine epidemiology impact and risk factors for pro-
gression Headache 200545(suppl 1)S3ndashS133 Knight T Steeves T Day L Lowerison M Jette N Pringsheim T Prevalence of tic
disorders a systematic review and meta-analysis Pediatr Neurol 20124777ndash904 Moreau JF Fink EL Hartman ME et al Hospitalizations of children with neurologic
disorders in the United States Pediatr Crit Care Med 201314801ndash8105 American Academy of Pediatrics Supporting the health care transition from ado-
lescence to adulthood in the medical home Pediatrics 2011128182ndash2006 Kivity S Lerman P Ariel R Danziger Y Mimouni M Shinnar S Long-term cognitive
outcomes of a cohort of children with cryptogenic infantile spasms treated with high-dose adrenocorticotropic hormone Epilepsia 200445255ndash262
7 Eisermann MM DeLaRaillere A Dellatolas G et al Infantile spasms in Down syn-drome effects of delayed anticonvulsive treatment Epilepsy Res 20035521ndash27
8 Goh S Kwiatkowski DJ Dorer DJ Thiele EA Infantile spasms and intellectualoutcomes in children with tuberous sclerosis complex Neurology 200565235ndash238
9 Riikonen R Long-term outcome in children with infantile spasms treated with vig-abatrin a cohort of 180 patients Epilepsia 201556807ndash809
10 Riikonen R A long-term follow-up study of 214 children with the syndrome ofinfantile spasms Neuropediatrics 19821314ndash23
11 Riikonen R Long-term outcome ofWest syndrome a study of adults with a history ofinfantile spasms Epilepsia 199637367ndash372
12 Widjaja E Go C McCoy B Snead O Neurodevelopmental outcome of infantilespasms a systematic review and meta-analysis Epilepsy Res 2015109155ndash162
13 Luthvigsson P Olafsson E Sigurdardottir S Hauser WA Epidemiologic features ofinfantile spasms in Iceland Epilepsia 199435802ndash805
14 Riikonen R Epidemiological data of West syndrome in Finland Brain Dev 200123539ndash541
15 Knupp KG Coryell J Nickels KC et al Response to treatment in a prospectivenational infantile spasms cohort Ann Neurol 201679475ndash484
16 Glauser T Shinnar S Gloss D et al Evidence-based guideline treatment of con-vulsive status epilepticus in children and adults report of the guideline committee ofthe American Epilepsy Society Epilepsy Curr 20161648ndash61
17 Brophy GM Bell R Claassen J et al Guidelines for the evaluation andmanagement ofstatus epilepticus Neurocrit Care 2012173ndash23
18 Cath D Hedderly T Ludolph A et al European clinical guidelines for Tourettesyndrome and other tic disorders part I assessment Eur Child Adolesc Psychiatry201120155ndash171
19 Knight T Steeves T Day L et al Prevalence of tic disorders a systematic review andmeta-analysis Pediatr Neurol 20124777ndash90
20 Tourette Syndrome Fact Sheet NIH Available at nindsnihgovdisorderstourettedetail_tourettehtm Accessed April 20 2016
21 Abu-Arafeh I Razak S Sivaraman B Graham C Prevalence of headache and migrainein children and adolescents a systematic review of population-based studies DevMedChild Neurol 2010521088ndash1097
22 Chiappedi M Mensi MM Termine C Balottin U Psychological therapy in adoles-cents with chronic daily headache Curr Pain Headache Rep 2016203
23 Lipton RB Nanack A Ricci JA et al Prevalence and burden of chronic migraine inadolescents results of the chronic daily headache in adolescents study (C-dAS)Headache 201151693ndash706
24 Powers SW Kashikar-Zuck SM Allen JR et al Cognitive behavioral therapy plusamitriptyline for chronic migraine in children and adolescents a randomized clinicaltrial JAMA 20133102622ndash2630
25 Faedda N Cerutti R Verdecchia P et al Behavioral management of headache inchildren and adolescents J Headache Pain 20161780
26 Bax M Goldstein M Rosenbaum P et al Executive Committee for the Definition ofCerebral Palsy Proposed definition and classification of cerebral palsy April 2005Dev Med Child Neurol 200547571ndash576
6 Neurology | Volume 90 Number 2 | January 9 2018 NeurologyorgN
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
27 Cerebral Palsy Fact Sheet NIH Available at nindsnihgovdisorderscerebral_palsydetail_cerebral_palsyhtm3104_2 Accessed September 3 2017
28 Shamsoddini A Amirsalari S Hollisaz MT Rahimnia A Khatibi-Aghda A Man-agement of spasticity in children with cerebral palsy Iranian J Pediatr 201424345ndash351
29 Michelson D Shevell M Sheer E et al Evidence report genetic and metabolic testingon children with global developmental delay Neurology 2011771629ndash1635
30 Kaufman L Ayub M Vincent JB The genetic basis of non-syndromic intellectualdisability a review J Neurodev Disord 20102182ndash209
31 Rauch A Hoyer J Guth S et al Diagnostic yield of various genetic approaches inpatients with unexplained developmental delay or mental retardation Am J MedGenet A 20061402063ndash2074
32 Riggs E Wain K Riethmaier D et al Chromosomal microarray impacts clinicalmanagement Clin Genet 201385147ndash153
33 Camfield P Gibson P Douglass L Strategies for transitioning to adult care for youthwith Lennox-Gastaut syndrome and related disorders Epilepsia 20115221ndash27
34 Brown L Camfield P Capers M et al The neurologistrsquos role in supporting transitionto adult health care Neurology 2016871ndash6
35 Camfield P Camfield C Transition to adult care for children with chronic neuro-logical disorders Ann Neurol 201169437ndash444
36 Borlot F Tellez-Zenteno JF Allen A Ali A Snead OC III Andrade DM Epilepsytransition challenges of caring for adults with childhood-onset seizures Epilepsia2014551659ndash1666
37 American Academy of Pediatrics Clinical report supporting the health care transitionfrom adolescence to adulthood in the medical home Pediatrics 2011128182ndash200
38 American Academy of Neurology Quality measurement manual 2014 Update Availableat aancomuploadedFilesWebsite_Library_AssetsDocuments3Practice_Manage-ment2Quality_Improvement1Quality_Measures2About_Quality_Measures20152002201120Process20Manual20Finalpdf Accessed March 9 2017
39 Go C Mackay M Weiss S et al Evidence-based guideline update medical treatmentof infantile spasms Neurology 2012781974ndash1980
40 Jones K Boyd J Go C et al Vigabatrin in the first line treatment of infantile spasmsEpilepsy Currents 201515533ndash534
41 Jones K Go C ACTH vs prednisolone in the treatment of infantile spasms postvigabatrin failure Epilepsy Currents 201414447ndash448
42 Jones K Go C Boyd J et al Vigabatrin as first-line treatment for infantile spasms notrelated to tuberous sclerosis complex Pediatr Neurol 201553141ndash145
43 Chapman KE Specchio N Shinnar S Holmes GL Seizing control of epileptic activitycan improve outcome Epilepsia 2015561482ndash1485
44 OrsquoCallaghan FJ Lux AL Darke K et al The effect of lead time to treatment andof age of onset on developmental outcome at 4 years in infantile spasms evi-dence from the United Kingdom Infantile Spasms Study Epilepsia 2011521359ndash1364
45 Trinka E Cock H Hesdorffer D et al A definition and classification of status epi-lepticus report of the ILAE Task Force on Classification of Status Epilepticus Epi-lepsia 2015561515ndash1523
NeurologyorgN Neurology | Volume 90 Number 2 | January 9 2018 7
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
DOI 101212WNL0000000000004806 published online December 15 2017Neurology
Anup D Patel Anne T Berg Lori Billinghurst et al summary
Quality improvement in neurology Child neurology quality measure set Executive
This information is current as of December 15 2017
ServicesUpdated Information amp
806fullhtmlhttpnneurologyorgcontentearly20171215WNL0000000000004including high resolution figures can be found at
Citations
806fullhtmlotherarticleshttpnneurologyorgcontentearly20171215WNL0000000000004This article has been cited by 1 HighWire-hosted articles
Subspecialty Collections
httpnneurologyorgcgicollectionall_pediatricAll Pediatric
httpnneurologyorgcgicollectionall_health_services_researchAll Health Services Researchfollowing collection(s) This article along with others on similar topics appears in the
Permissions amp Licensing
httpnneurologyorgmiscaboutxhtmlpermissionsits entirety can be found online atInformation about reproducing this article in parts (figurestables) or in
Reprints
httpnneurologyorgmiscaddirxhtmlreprintsusInformation about ordering reprints can be found online
Neurology All rights reserved Print ISSN 0028-3878 Online ISSN 1526-632X1951 it is now a weekly with 48 issues per year Copyright Copyright copy 2017 American Academy of
reg is the official journal of the American Academy of Neurology Published continuously sinceNeurology
energy contributions and work that supported the de-velopment of this article Jeffrey Buchhalter MD FAAN(Chair Child Neurology Society) Anup Patel MD FAAN(Chair American Academy of Neurology) Karen Ballaban-Gil MD (American Academy of Neurology) Anne BergPhD (American Academy of Neurology) Daniel Fain MDFAAN (American Academy of Neurology) Cynthia KeatorMD (American Academy of Neurology) Sanjeev KothareMD (American Academy of Neurology) Gogi Kumar MD(American Academy of Neurology) Migvis MonduyMD (American Academy of Neurology) Diego Morita MD(American Academy of Neurology) Zachary Grinspan MDMS (American Academy of Pediatrics Child NeurologySociety) Erin Fecske MSN RN CNRN CPNP (AmericanAssociation of Neuroscience Nurses) Amy Houtrow MDPhD MPH (American Academy of Physical Medicine andRehabilitation) Kevin Chapman MD (American EpilepsySociety) Christina Szperka MD (American Headache Soci-ety) Lynn Jeffries PT DPT PhD PCS (American PhysicalTherapy Association) Tim Feyma MD (Child NeurologySociety) Kiran Maski MD (Child Neurology Society) LisaMeunier (Epilepsy Foundation) Lori Billinghurst MD MScFRCPC (independent) Ann Yeh MD (independent) DavidMichelsonMD (Facilitator AmericanAcademy ofNeurology)M Cristina Victorio MD (Facilitator American Academyof Neurology) Amy Bennett JD (American Academy ofNeurology staff) Gina Gjorvad (American Academy ofNeurology staff) Erin Lee (American Academy of Neurologystaff) Becky Schierman MPH (American Academy ofNeurology staff)
Study fundingNo targeted funding reported
DisclosureA Patel receives research grants from the Pediatric EpilepsyResearch Foundation Upsher-Smith GW Pharmaceuticalsand LivaNova He consults for GW Pharmaceuticals andLivaNova He is on a scientific advisory board for UCBPharma and Supernus A Berg and L Billinghurst report nodisclosures relevant to the manuscript D Fain is a sub-investigator for the following study as of September 6 2016 ARandomized Double-Blind Placebo-Controlled Study toEvaluate the Efficacy and Safety of Sage-547 Injection in theTreatment of Subjects with Super-Refractory Status Epi-lepticus Protocol number 547-SSE-301 E Fecske andT Feyma report no disclosures relevant to the manuscriptZ Grinspan receives funding from the Centers for DiseaseControl and Prevention the Pediatric Epilepsy ResearchFoundation and the Nanette Laitman Clinical Scholars Pro-gram A Houtrow S Kothare and G Kumar report no dis-closures relevant to the manuscript E Lee is an employee ofthe American Academy of Neurology M Monduy andD Morita report no disclosures relevant to the manuscriptC Szperka received funding from Pfizer andMiles for MigraineM Victorio reports no disclosures relevant to themanuscript AYeh receives research funding from the NMSS CMSC IORM
SCN CBMH Chase an Idea SickKids Foundation Rare Dis-eases Foundation MS Scientific Foundation (Canada)MSSociety McLaughlin Centre and Mario Battaglia FoundationShe has received unrestricted funds to run an educationalsymposium from theGuthy Jackson Charitable Foundation andTeva She has served on a scientific advisory board for neuro-toxicity for Juno She serves as a relapse adjudicator for ACI Shehas received honorarium for a talk from Novartis J Buchhalteris on the Scientific Advisory Board of the Charlie FoundationHe receives research funding from the Alberta ChildrenrsquosHospital Research Institute and the Department of PediatricsUniversity of Calgary Alberta Canada Go to NeurologyorgNfor full disclosures
Received May 17 2017 Accepted in final form September 25 2017
References1 Lipton RB Bigal ME The epidemiology of migraine Am J Med 2005118(suppl 1)
3Sndash10S2 Lipton RB Bigal ME Migraine epidemiology impact and risk factors for pro-
gression Headache 200545(suppl 1)S3ndashS133 Knight T Steeves T Day L Lowerison M Jette N Pringsheim T Prevalence of tic
disorders a systematic review and meta-analysis Pediatr Neurol 20124777ndash904 Moreau JF Fink EL Hartman ME et al Hospitalizations of children with neurologic
disorders in the United States Pediatr Crit Care Med 201314801ndash8105 American Academy of Pediatrics Supporting the health care transition from ado-
lescence to adulthood in the medical home Pediatrics 2011128182ndash2006 Kivity S Lerman P Ariel R Danziger Y Mimouni M Shinnar S Long-term cognitive
outcomes of a cohort of children with cryptogenic infantile spasms treated with high-dose adrenocorticotropic hormone Epilepsia 200445255ndash262
7 Eisermann MM DeLaRaillere A Dellatolas G et al Infantile spasms in Down syn-drome effects of delayed anticonvulsive treatment Epilepsy Res 20035521ndash27
8 Goh S Kwiatkowski DJ Dorer DJ Thiele EA Infantile spasms and intellectualoutcomes in children with tuberous sclerosis complex Neurology 200565235ndash238
9 Riikonen R Long-term outcome in children with infantile spasms treated with vig-abatrin a cohort of 180 patients Epilepsia 201556807ndash809
10 Riikonen R A long-term follow-up study of 214 children with the syndrome ofinfantile spasms Neuropediatrics 19821314ndash23
11 Riikonen R Long-term outcome ofWest syndrome a study of adults with a history ofinfantile spasms Epilepsia 199637367ndash372
12 Widjaja E Go C McCoy B Snead O Neurodevelopmental outcome of infantilespasms a systematic review and meta-analysis Epilepsy Res 2015109155ndash162
13 Luthvigsson P Olafsson E Sigurdardottir S Hauser WA Epidemiologic features ofinfantile spasms in Iceland Epilepsia 199435802ndash805
14 Riikonen R Epidemiological data of West syndrome in Finland Brain Dev 200123539ndash541
15 Knupp KG Coryell J Nickels KC et al Response to treatment in a prospectivenational infantile spasms cohort Ann Neurol 201679475ndash484
16 Glauser T Shinnar S Gloss D et al Evidence-based guideline treatment of con-vulsive status epilepticus in children and adults report of the guideline committee ofthe American Epilepsy Society Epilepsy Curr 20161648ndash61
17 Brophy GM Bell R Claassen J et al Guidelines for the evaluation andmanagement ofstatus epilepticus Neurocrit Care 2012173ndash23
18 Cath D Hedderly T Ludolph A et al European clinical guidelines for Tourettesyndrome and other tic disorders part I assessment Eur Child Adolesc Psychiatry201120155ndash171
19 Knight T Steeves T Day L et al Prevalence of tic disorders a systematic review andmeta-analysis Pediatr Neurol 20124777ndash90
20 Tourette Syndrome Fact Sheet NIH Available at nindsnihgovdisorderstourettedetail_tourettehtm Accessed April 20 2016
21 Abu-Arafeh I Razak S Sivaraman B Graham C Prevalence of headache and migrainein children and adolescents a systematic review of population-based studies DevMedChild Neurol 2010521088ndash1097
22 Chiappedi M Mensi MM Termine C Balottin U Psychological therapy in adoles-cents with chronic daily headache Curr Pain Headache Rep 2016203
23 Lipton RB Nanack A Ricci JA et al Prevalence and burden of chronic migraine inadolescents results of the chronic daily headache in adolescents study (C-dAS)Headache 201151693ndash706
24 Powers SW Kashikar-Zuck SM Allen JR et al Cognitive behavioral therapy plusamitriptyline for chronic migraine in children and adolescents a randomized clinicaltrial JAMA 20133102622ndash2630
25 Faedda N Cerutti R Verdecchia P et al Behavioral management of headache inchildren and adolescents J Headache Pain 20161780
26 Bax M Goldstein M Rosenbaum P et al Executive Committee for the Definition ofCerebral Palsy Proposed definition and classification of cerebral palsy April 2005Dev Med Child Neurol 200547571ndash576
6 Neurology | Volume 90 Number 2 | January 9 2018 NeurologyorgN
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
27 Cerebral Palsy Fact Sheet NIH Available at nindsnihgovdisorderscerebral_palsydetail_cerebral_palsyhtm3104_2 Accessed September 3 2017
28 Shamsoddini A Amirsalari S Hollisaz MT Rahimnia A Khatibi-Aghda A Man-agement of spasticity in children with cerebral palsy Iranian J Pediatr 201424345ndash351
29 Michelson D Shevell M Sheer E et al Evidence report genetic and metabolic testingon children with global developmental delay Neurology 2011771629ndash1635
30 Kaufman L Ayub M Vincent JB The genetic basis of non-syndromic intellectualdisability a review J Neurodev Disord 20102182ndash209
31 Rauch A Hoyer J Guth S et al Diagnostic yield of various genetic approaches inpatients with unexplained developmental delay or mental retardation Am J MedGenet A 20061402063ndash2074
32 Riggs E Wain K Riethmaier D et al Chromosomal microarray impacts clinicalmanagement Clin Genet 201385147ndash153
33 Camfield P Gibson P Douglass L Strategies for transitioning to adult care for youthwith Lennox-Gastaut syndrome and related disorders Epilepsia 20115221ndash27
34 Brown L Camfield P Capers M et al The neurologistrsquos role in supporting transitionto adult health care Neurology 2016871ndash6
35 Camfield P Camfield C Transition to adult care for children with chronic neuro-logical disorders Ann Neurol 201169437ndash444
36 Borlot F Tellez-Zenteno JF Allen A Ali A Snead OC III Andrade DM Epilepsytransition challenges of caring for adults with childhood-onset seizures Epilepsia2014551659ndash1666
37 American Academy of Pediatrics Clinical report supporting the health care transitionfrom adolescence to adulthood in the medical home Pediatrics 2011128182ndash200
38 American Academy of Neurology Quality measurement manual 2014 Update Availableat aancomuploadedFilesWebsite_Library_AssetsDocuments3Practice_Manage-ment2Quality_Improvement1Quality_Measures2About_Quality_Measures20152002201120Process20Manual20Finalpdf Accessed March 9 2017
39 Go C Mackay M Weiss S et al Evidence-based guideline update medical treatmentof infantile spasms Neurology 2012781974ndash1980
40 Jones K Boyd J Go C et al Vigabatrin in the first line treatment of infantile spasmsEpilepsy Currents 201515533ndash534
41 Jones K Go C ACTH vs prednisolone in the treatment of infantile spasms postvigabatrin failure Epilepsy Currents 201414447ndash448
42 Jones K Go C Boyd J et al Vigabatrin as first-line treatment for infantile spasms notrelated to tuberous sclerosis complex Pediatr Neurol 201553141ndash145
43 Chapman KE Specchio N Shinnar S Holmes GL Seizing control of epileptic activitycan improve outcome Epilepsia 2015561482ndash1485
44 OrsquoCallaghan FJ Lux AL Darke K et al The effect of lead time to treatment andof age of onset on developmental outcome at 4 years in infantile spasms evi-dence from the United Kingdom Infantile Spasms Study Epilepsia 2011521359ndash1364
45 Trinka E Cock H Hesdorffer D et al A definition and classification of status epi-lepticus report of the ILAE Task Force on Classification of Status Epilepticus Epi-lepsia 2015561515ndash1523
NeurologyorgN Neurology | Volume 90 Number 2 | January 9 2018 7
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
DOI 101212WNL0000000000004806 published online December 15 2017Neurology
Anup D Patel Anne T Berg Lori Billinghurst et al summary
Quality improvement in neurology Child neurology quality measure set Executive
This information is current as of December 15 2017
ServicesUpdated Information amp
806fullhtmlhttpnneurologyorgcontentearly20171215WNL0000000000004including high resolution figures can be found at
Citations
806fullhtmlotherarticleshttpnneurologyorgcontentearly20171215WNL0000000000004This article has been cited by 1 HighWire-hosted articles
Subspecialty Collections
httpnneurologyorgcgicollectionall_pediatricAll Pediatric
httpnneurologyorgcgicollectionall_health_services_researchAll Health Services Researchfollowing collection(s) This article along with others on similar topics appears in the
Permissions amp Licensing
httpnneurologyorgmiscaboutxhtmlpermissionsits entirety can be found online atInformation about reproducing this article in parts (figurestables) or in
Reprints
httpnneurologyorgmiscaddirxhtmlreprintsusInformation about ordering reprints can be found online
Neurology All rights reserved Print ISSN 0028-3878 Online ISSN 1526-632X1951 it is now a weekly with 48 issues per year Copyright Copyright copy 2017 American Academy of
reg is the official journal of the American Academy of Neurology Published continuously sinceNeurology
27 Cerebral Palsy Fact Sheet NIH Available at nindsnihgovdisorderscerebral_palsydetail_cerebral_palsyhtm3104_2 Accessed September 3 2017
28 Shamsoddini A Amirsalari S Hollisaz MT Rahimnia A Khatibi-Aghda A Man-agement of spasticity in children with cerebral palsy Iranian J Pediatr 201424345ndash351
29 Michelson D Shevell M Sheer E et al Evidence report genetic and metabolic testingon children with global developmental delay Neurology 2011771629ndash1635
30 Kaufman L Ayub M Vincent JB The genetic basis of non-syndromic intellectualdisability a review J Neurodev Disord 20102182ndash209
31 Rauch A Hoyer J Guth S et al Diagnostic yield of various genetic approaches inpatients with unexplained developmental delay or mental retardation Am J MedGenet A 20061402063ndash2074
32 Riggs E Wain K Riethmaier D et al Chromosomal microarray impacts clinicalmanagement Clin Genet 201385147ndash153
33 Camfield P Gibson P Douglass L Strategies for transitioning to adult care for youthwith Lennox-Gastaut syndrome and related disorders Epilepsia 20115221ndash27
34 Brown L Camfield P Capers M et al The neurologistrsquos role in supporting transitionto adult health care Neurology 2016871ndash6
35 Camfield P Camfield C Transition to adult care for children with chronic neuro-logical disorders Ann Neurol 201169437ndash444
36 Borlot F Tellez-Zenteno JF Allen A Ali A Snead OC III Andrade DM Epilepsytransition challenges of caring for adults with childhood-onset seizures Epilepsia2014551659ndash1666
37 American Academy of Pediatrics Clinical report supporting the health care transitionfrom adolescence to adulthood in the medical home Pediatrics 2011128182ndash200
38 American Academy of Neurology Quality measurement manual 2014 Update Availableat aancomuploadedFilesWebsite_Library_AssetsDocuments3Practice_Manage-ment2Quality_Improvement1Quality_Measures2About_Quality_Measures20152002201120Process20Manual20Finalpdf Accessed March 9 2017
39 Go C Mackay M Weiss S et al Evidence-based guideline update medical treatmentof infantile spasms Neurology 2012781974ndash1980
40 Jones K Boyd J Go C et al Vigabatrin in the first line treatment of infantile spasmsEpilepsy Currents 201515533ndash534
41 Jones K Go C ACTH vs prednisolone in the treatment of infantile spasms postvigabatrin failure Epilepsy Currents 201414447ndash448
42 Jones K Go C Boyd J et al Vigabatrin as first-line treatment for infantile spasms notrelated to tuberous sclerosis complex Pediatr Neurol 201553141ndash145
43 Chapman KE Specchio N Shinnar S Holmes GL Seizing control of epileptic activitycan improve outcome Epilepsia 2015561482ndash1485
44 OrsquoCallaghan FJ Lux AL Darke K et al The effect of lead time to treatment andof age of onset on developmental outcome at 4 years in infantile spasms evi-dence from the United Kingdom Infantile Spasms Study Epilepsia 2011521359ndash1364
45 Trinka E Cock H Hesdorffer D et al A definition and classification of status epi-lepticus report of the ILAE Task Force on Classification of Status Epilepticus Epi-lepsia 2015561515ndash1523
NeurologyorgN Neurology | Volume 90 Number 2 | January 9 2018 7
Copyright ordf 2017 American Academy of Neurology Unauthorized reproduction of this article is prohibited
DOI 101212WNL0000000000004806 published online December 15 2017Neurology
Anup D Patel Anne T Berg Lori Billinghurst et al summary
Quality improvement in neurology Child neurology quality measure set Executive
This information is current as of December 15 2017
ServicesUpdated Information amp
806fullhtmlhttpnneurologyorgcontentearly20171215WNL0000000000004including high resolution figures can be found at
Citations
806fullhtmlotherarticleshttpnneurologyorgcontentearly20171215WNL0000000000004This article has been cited by 1 HighWire-hosted articles
Subspecialty Collections
httpnneurologyorgcgicollectionall_pediatricAll Pediatric
httpnneurologyorgcgicollectionall_health_services_researchAll Health Services Researchfollowing collection(s) This article along with others on similar topics appears in the
Permissions amp Licensing
httpnneurologyorgmiscaboutxhtmlpermissionsits entirety can be found online atInformation about reproducing this article in parts (figurestables) or in
Reprints
httpnneurologyorgmiscaddirxhtmlreprintsusInformation about ordering reprints can be found online
Neurology All rights reserved Print ISSN 0028-3878 Online ISSN 1526-632X1951 it is now a weekly with 48 issues per year Copyright Copyright copy 2017 American Academy of
reg is the official journal of the American Academy of Neurology Published continuously sinceNeurology
DOI 101212WNL0000000000004806 published online December 15 2017Neurology
Anup D Patel Anne T Berg Lori Billinghurst et al summary
Quality improvement in neurology Child neurology quality measure set Executive
This information is current as of December 15 2017
ServicesUpdated Information amp
806fullhtmlhttpnneurologyorgcontentearly20171215WNL0000000000004including high resolution figures can be found at
Citations
806fullhtmlotherarticleshttpnneurologyorgcontentearly20171215WNL0000000000004This article has been cited by 1 HighWire-hosted articles
Subspecialty Collections
httpnneurologyorgcgicollectionall_pediatricAll Pediatric
httpnneurologyorgcgicollectionall_health_services_researchAll Health Services Researchfollowing collection(s) This article along with others on similar topics appears in the
Permissions amp Licensing
httpnneurologyorgmiscaboutxhtmlpermissionsits entirety can be found online atInformation about reproducing this article in parts (figurestables) or in
Reprints
httpnneurologyorgmiscaddirxhtmlreprintsusInformation about ordering reprints can be found online
Neurology All rights reserved Print ISSN 0028-3878 Online ISSN 1526-632X1951 it is now a weekly with 48 issues per year Copyright Copyright copy 2017 American Academy of
reg is the official journal of the American Academy of Neurology Published continuously sinceNeurology