it’s not all about migraine!az-ns.org/presentations/its-not-all-about-migraine.pdf13 year old with...
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E R I C H A S T R I T E R , M D F A H S
B A N N E R P E D I A T R I C S P E C I A L I S T S
C A R D O N C H I L D R E N ’ S M E D I C A L C E N T E R
D E C E M B E R 6 T H , 2 0 1 6
It’s not all about Migraine!
MY PROCESS
THE SAGA CONTINUED
Program Disclaimer: The accuracy and utility of the
materials presented are based on the International
Classification of Headache Disorders, 2nd edition (ICHD-
II), statements made will be evidence-based, the
limitations being due to the nature of trials in children,
most of the evidence comes from adult studies but clinical
evidence across the pediatric headache community will be
discussed
Conflict of Interest: There is no conflict of interest at this
time
Objectives
Describe a typical diagnostic path used by neurologists obtaining proper primary headache diagnosis other than migraine
Understand the utility of preventative or abortive medications, diagnostic tests, and psychological/bio-behavioral approaches in management of diagnosis
Discuss the timing of when to refer to a specialist
Migraine Criteria- ICHD-II
At least 5 attacks, lasting 1 to 72 hours
Headache has at least 2 of the following:
• Unilateral location, may be bilateral, frontotemporal (not occipital)
• Pulsating quality (throbbing)
• Moderate or severe pain intensity
• Aggravated by routine physical activity
• at least 1 of the following:
Nausea and/or vomiting.
Photophobia and phonophobia
may be inferred from the child’s behavior
• Not attributed to another disorder
17 year old male
Frequent headaches that began in august (with school)
2-3/month, 1 hour to 5 days
Global, squeezing, moderately intense, did not interfere with his activity
No nausea, vomiting, phonophobia, but had photophobia
Worked 4 nights/week at fast food establishment to save money for college
PMH, FH, SH and ROS were benign, general and neurologic exam were normal
What was the most likely diagnosis?
What diagnostic studies were indicated?
What management options were appropriate?
Tension Type Headache
30 minutes to 7 days,
bilateral, pressing/tightening (non-pulsatile)
mild to moderate intensity,
not aggravated by physical exertion
Associated with:
lack of associated features
may have pericranial muscle tenderness
nausea/vomiting do not occur
no more than 1 of photophobia/phonophobia and it is not prominent
Tension Type Headache
Most common type worldwide
Rarely interferes with function
Infrequently present if episodic
Most common reasons buying OTC meds
Little known about pathogenesis of tension-type headache
1-year prevalence ranges from 40-80%
Slightly more common in females
Tension Type Headache
Differential: episodic migraine, cervicogenic headache, and secondary causes of headache
If presents with chronic tension type headache: neuroimaging is warranted to rule out space occupying lesions
Cervicogenic HA-strictly unilateral headache
Tension Type Headache
Treatment-lifestyle changes (stress reduction and management, relaxation therapy, massage, cognitive behavioral therapy
Abortives: NSAIDS, combination products with caffeine if caffeine naïve
Preventatives if > 10 days/month, amitriptyline or nortriptyline
Tension Type Headache
What was the most likely diagnosis?
What diagnostic studies were indicated?
What management options were appropriate?
13 year old female
March 2011: flu-like illness (mom thought), headaches began and have been daily
Bilateral, continuous moderate pain, she had difficulty describing quality (feel tightening, throbbing, pulsating, worse with movement)
Some nausea, photophobia and phonophobia, but they were not prominent
No autonomic features
No systemic or neurologic symptoms
13 year old female
Labs from nearly 2 years ago, mono positive, and then a year after the fact West Nile Virus was positive
MRI, MRA, MRV all were negative
Hematologic workup was negative
LP was normal pressure, no infection
13 year old female
What diagnosis could this be?
What needs done in this patient?
What treatment is suggested in this patient?
New Daily Persistant Headache
Subacute onset over 72 hours of daily unremitting headache
Resemble CTTH, or CM, but is chronic at onset
Diagnosis of exclusion: neuroimaging, hematologic workup required to rule out secondary causes
May resolve on own or be refractory to treatment
New Daily Persistant Headache
Most common in adolescents/young adults
~10% of patients in tertiary headache clinics
Underlying etiology unknown (30% report recent flu-like illness at time of onset)
Two clinical subtypes-
benign self limited form
refractory form resistant to aggressive therapy
New Daily Persistant Headache
Bilateral, continuous moderate pain(can be mild or severe)
Tightening/throbbing/pulsating, and may be aggravated by physical exercise
Migraine features (N/V/P/P) but typically not most prominent features
No associated trigeminal autonomic features (lacrimation/conjunctival injection)
No systemic or neurologic symptoms
New Daily Persistant Headache
Differential includes CTTH, CM and secondary causes of headaches
Can resemble spontaneous intracranial hypotension (dural tear or leak) if positional component overlooked in history
Can resemble pseudotumor cerebri (idiopathic intracranial hypertension)
New Daily Persistant Headache
Labs: CBC (rule out chronic anemia or infection), TSH (rule out hypothyroidism), CRP/ESR (rule out temporal arteritis)
MRI brain: space occupying lesion, hydrocephalus,
MRI w/GAD: spontaneous intracranial hypotension
MRV: cerebral venous sinus thrombosis
LP: increased/decreased CSF pressure or chronic meningitis
New Daily Persistant Headache
Treatment- no known effective acute or prophylactic therapy
Standard acute/preventative for CTTH, CM could be attempted on trial and error basis
Doxycycline, Singulair have been used
Beware: Medication overuse headache complicating picture
New Daily Persistant Headache
What diagnosis could this be?
What needs done in this patient?
What treatment is suggested in this patient?
14 year old male, eye pain
Sharp excruciating right sided orbital and temporal pain
Tearing, reddening of eye, ringing in right ear
About 20 minutes, 8 times/day, but 1 lasted 3 hours
He feels agitated, awakening nightly during baseball season every year
Nausea occurs, and so does photophobia
Between episodes has lesser pain and takes 800 mg ibuprofen regularly which helps
14 year old male, eye pain
What is his diagnosis?
What investigations need done?
How would you treat this entity?
Cluster Headache
Excrutiating strictly unilateral headache, last about 1 hour (15min-3hours), and may recur up to 8/day usually seasonal
Associated with cranial autonomic symptoms ipsilateral to pain, can be bilateral
20% have CCH when headaches continue for more than 1 year without remission of > 4 weeks
Cluster Headache
Co-Morbid: Tobacco use, OSA
MRI brain: intracranial lesions particularly in pituitary and parasellar regions
Treatments:
100% oxygen
Intranasal or parenteral triptans
Corticosteroids
Verapamil
Cluster Headache
Prototypical TAC-Trigeminal Autonomic Cephalgias; first division of trigeminal nerve, accompanying ipsilateral autonomic features (lacrimation/ conjuctival injection/rhinorrhea)
80% have cluster 1-3 months, typically 1-2 times per year with remissions in between
Nocturnal attacks typical, but daytime attacks occur, usually near same time daily
Cluster Headache
Triggers: alcohol and high altitude
Strictly unilateral, maximal around or above orbit, may begin or become referred to the temporal, lower facial, or occipital region
Extremely severe, piercing, boring, stabbing peaking within 3-5 minutes lasting average 1 hour (15 min- 3 hour)
Cluster Headache
> 90% agitation
Autonomic features: lacrimation, rhinorrhea, conjunctival injection, ptosis, miosis, facial or periorbital edema
Autonomic features may be bilateral, but are prominent ipsilateral to pain
Cluster Headache
Migrainous symptoms (N/V/P/P)
Can have unilateral Photo/phonophobia
Interparoxysmal pain and allodynia (abnormal pain response to normal stimulus) may occur in more than 1/3 of patients with any of the TACs
Medication Overuse may be responsible the interparoxysmal pain
Cluster Headache
PE may show persistant Horner syndrome if had recurrent attacks for years
MRI brain: special attention to pituitary and parasellar regions for mimickers of TACs
Overnight polysomnography if features of OSA are present
Cluster Headache
Treatment
Acute- 100% oxygen 7-15L/min with closed facemask for 15 minutes
Triptans-sumatriptan 6mg SC, sumatriptan 20 mg NS, zolmitriptan 5 mg NS
Short term prevention- steroid taper 60 mg decrease by 10 mg every 2-3 days
Occipital nerve blockade 2.5 mL bupivacaine with 20 mg methylprednisolone
Cluster Headache
Preventatives
Verapamil 80 mg TID up to 320 mg TID (check ECG after each dose increase).
Long term side effects: gingival hyperplasia, constipation, and peripheral edema)
Cluster Headache
What else could be his diagnosis?
What investigations need done?
How would you treat this entity?
13 year old with migraine
Headaches since she was 6, diagnosed with migraine and nothing seems to be helping
Pain is all day long, sharp, stabbing, usually around the temples, mostly on left side
Both eyes get red, ears ringing bilaterally
Sensitive to light and sound out of the left ear and eye
Nausea present, no vomiting
Ibuprofen helps but it comes back
13 year old with migraine
PMH, FH, SH, ROS and physical exam all normal
When asked about all day long headache, she said it came and went throughout the day
Further questioning revealed that is came and went around 20-30 minutes, but happened 8-20 times per day
In between she was head pain free
13 year old with migraine
What could be her diagnosis?
What investigations need done?
How would you treat this entity?
Paroxysmal Hemicrania
Similar to CH however shorter and more frequent attacks
MRI brain with coronal GAD sequences of pituitary for all suspected paroxysmal hemicrania
Responds rapidly AND completely to indomethacin
Paroxysmal Hemicrania
Slight female predominance
Late adolescence to early adulthood mean age 36 (1 year -81 years reported)
Typically unilateral (bilateral reported though)
Located maximally in V1 region, however may occur in parietal and temporal regions
Paroxysmal Hemicrania
Severe intensity, throbbing, pressure, stabbing or boring
Attacks 2-30 minutes, 1-40/day, average 5-15/day
Paroxysmal Hemicrania
Autonomic features: ipsilateral lacrimation, nasal congestion most common, but can inlude rhinorrhea, conjunctival injection, eyelid edema, ptosis, miosis, forehead or facial sweating (>50% of patients), ear fullness sensation in 30% of patients
Migraine features: Photophobia/phonophobia 66% of patients, may be unilateral
Paroxysmal Hemicrania
Nausea/vomiting 33% of patients
Motion sensitivity 50%
Agitation/Restlessness in 80%
Spontaneous attacks, no nocturnal predominance
Differential: cluster headache, trigeminal neuralgia, SUNCT syndrome, and hemicrania continua
Paroxysmal Hemicrania
Dull inter-ictal pain, may resemble hemicrania continua, but hemicrania continua exacerbations are longer lasting with less autonomic features
Secondary causes have been reported with lesions in pituitary and posterior fossa
Treatment: Indomethacin
Paroxysmal Hemicrania
Titrate up to 75 mg TID, protect mucosal lining due to gastric side effects, if poorly tolerated cox-2 inhibitors, ASA, topiramate, or gabapentin may be tried
Occipital nerve blockade may help
May last years or decades
Some have spontaneous remission
Paroxysmal Hemicrania
What else could be her diagnosis?
What investigations need done?
How would you treat this entity?
15 year old excruciating pains
4 year history of headaches behind right eye that last all day
Severe to excruciating, stabbing on right temple/eye, tearing in right eye, injection in right eye
Able to trigger it combing hair occasionally, but not always
Nothing over the counter seemed to help
Amitriptyline had been tried unsuccessfully
15 year old excruciating pains
Further questioning revealed he had pain that came and went throughout day
The longest pain was 3 minutes and the shortest pain was 5 seconds of stabbing
He estimated the range of episodes throughout the day were around 60-70 times/day
PMH/FH/SH/ ROS and exam were normal
15 year old excruciating pains
What could be his diagnosis?
What investigations need done?
How would you treat this entity?
SUNCT Syndrome
Short lasting Unilateral Neuralgiform Headache attacks with Conjunctival Tearing
Strictly unilateral, severe, stabbing, shooting, lancinating, burning, V1 distribution occur with conjunctival injection and tearing
5 - 240 seconds, in between attacks pain free
Frequent up to 200/day attacks
Slight male predominance
Age 35-65 years (10-77 years)
Natural history-last years, decades or lifelong
SUNCT Syndrome
Migrainous features are not uncommon especially unilateral photophobia
Autonomic symptoms are frequently seen including rhinorrhea, nasal congestion, eyelid edema, ptosis, miosis, facial redness, conjunctival tearing obviously
May be triggered by tactile stimuli, most commonly mastication and trigeminal innervated areas
SUNCT Syndrome
Brain MRI looking at pituitary, parasellar region, and brainstem in all patients with suspected SUNCT syndrome
Anticonvulsants: lamotrigine, gabapentin, topiramate, and carbamazepine.
Parenteral lidocaine: effective in most
Surgical procedures- mixed results
Percutaneous trigeminal ganglion rhizolysis
Trigeminal root microvascular decompression
Hypothalamic deep brain stimulation
SUNCT Syndrome
What else could be his diagnosis?
What investigations need done?
How would you treat this entity?
14 year old girl with stabbing pain
4 month headache history
Occasional stabbing in random locations, most in temple and frontal area that lasted 1-10 seconds, and up to 10 a day, then some days without any pain
No migraine features
No autonomic features
Occasionally would have a true migraine, lasting hours, worse with movement, associated with N/V/P/P responds to naproxen
14 year old girl with stabbing pain
What could be her diagnosis?
What investigations need done?
How would you treat this entity?
Primary Stabbing Headache
Alias: ophthalmodynia periodica, ice pick headache, jabs and jolts syndrome, idiopathic stabbing headache
Ultra short paroxysms of stabbing pain (1-10s) unilateral ophthalmic division most common, however anywhere on head may occur, 1-50 times throughout day and evening
Onset 12-70 (mean 47 years), female predominance
Primary Stabbing Headache
Differential includes SUNCT (attacks longer) or Trigeminal Neuralgia (more V2/V3) or secondary causes
Frequent attacks: indomethacin is treatment of choice as preventative
Frequently associated with migraine (40%), tension type headache and the TACs
Secondary causes: meningiomas, pituitary tumors, giant cell arteritis, cranial and ocular trauma, herpes zoster, and elevated intraocular pressure
Primary Stabbing Headache
Frequently associated with migraine (40%), tension type headache and the TACs
Secondary causes: meningiomas, pituitary tumors, giant cell arteritis, cranial and ocular trauma, herpes zoster, and elevated intraocular pressure
Primary Stabbing Headache
Usually unilateral
Attacks from 1-50 attacks/day
Most attacks throughout day and evening
Differential includes SUNCT (attacks longer) or Trigeminal Neuralgia (more V2/V3) or secondary causes
Primary Stabbing Headache
Investigations usually not necessary unless suspicious for secondary causes
Prophylaxis rarely required
Indomethacin 25-75mg TID
Melatonin 3-12 mg/day
Gabapentin 400 mg BID
Primary Stabbing Headache
What else could be her diagnosis?
What investigations need done?
How or would you treat this entity?
Tests used
Used DSM-IV to make diagnosis
CBCL-child behavior checklist age weighted
YSR-youth self report
SCARED-the screen for Child Anxiety Related Disorders
CDI-Children’s Depression Inventory
CPRS-R; Conner’s Parent Rating Scale-Revised
CBCL-child behavior checklist
Filled out by parents to assess emotional/behavioral
problems of children and adolescents
Identifies two types of problems- internalizing and
externalizing
Internalizing- anxiety, depression, social withdrawal, and
somatic complaints
Externalizing- aggression and antisocial behavior
SCARED-screen for childhood anxiety related
disorders Self administered
41 questions over last three months of feelings of anxiety
Score >39 indicates clinical impairment
CDI-children’s depression inventory
Derived from Beck’s depression Scale
Assesses severity of depression symptoms in prior two
weeks
Age 7-17
19 points or higher discriminates risk for depression
CPSR-Conner’s Parent Rating Scale
Self administered
80 questions about behavior during last month
Ages 3-17
Scores> 65 indicate clinical impairment
Results
Children with headaches had significant internalizing and
externalizing problems compared with control 63% and
27%
No difference between migraine and tension type found
26% had positive comorbidity with the headache of
anxiety and mood disorders.
One out of three headache patients needed particular therapy with emotional and
behavioral problems
Headache Toolbox
Proven Behavioral Therapies
Relaxation training
Temperature biofeedback (hand warming) combined with relaxation
training
Electromyographic (EMG) biofeedback (for muscle tension
reduction
Cognitive Behavior Therapy
Complementary Therapies
Accupuncture/accupressure
Chiropractic therapy
Hypnosis and physical therapy
Cognitive Behavioral Therapy
Highly effective
Average rate of reduction in headaches compared to
controls 68% vs. 20%
Improvement was seen in baseline coping skills, social
support, physiologic measures at rest and in response to
stress.
Noncompliance
Prevalent in headache patients
Simplifying medication strategies
Screening and management of psychiatric co-morbidities
Enabling patient to have self-efficacy and take ownership
of their headaches is key
Website for relaxation: www.dawnbuse.com
Natural Remedies for headaches
LipiGesic- sublingual feverfew/ginger appears safe and
effective as first line abortive in migraine patients at the
onset of a pre-severe migraine (most common side effect
is nausea of gagging)
Exercise-systematic review on the literature concluded it
was promising but adherence to headache research
guidelines in a study needed.
�Natural Remedies for headaches
Thirty studies reviewed on 6 nutraceuticals
Butterbur-showed reduction and liver toxicity
Riboflavin-400 mg showed marked reduction
Ginkgolide B-60-80 mg showed marked reduction
Magnesium- 300 mg showed marked reduction
Coenzyme Q10- 100 mg showed marked reduction
Polyunsaturated fatty acids-Marine ester concentrate- showed
marked reduction
Studies showed strong evidence but level of evidence is still low in
all-more studies are needed.
When to Refer to Headache Specialist
When not responding to OTC medications, preventatives or abortives
Frequency of headache increasing or disability increasing
When something other than migraine suspected, but uncomfortable treating it
When you would like help with the time-consuming patient
When patient has chronic migraine or chronic daily headache
When to Refer to Headache Specialist
Headache specialist requested by parent/patient
Role of Pediatric Headache specialist-
Taking time to establish proper diagnosis
Get patient on a good preventative
Get patient on good rescue medication
Decreasing headache disability
Returning improved pediatric patient to their primary care provider
References
Baillie LE, Gabriele JM, Penzien DB. A systematic review of behavioral headache interventions with an aerobic exercise component. Headache 2014;54(1): 40-53.
Penzien DB. Stress management for migraine: recent research and commentary. Headaches 2009;49(9):1395-1398.
Rains JC, Penzien DB, Lipchick GL. Behavioral facilitation of medical treatment of headache: implications of noncompliance and strategies for improving adherence. Headache 2006;46 (suppl 3):S142-S143.
Seng EK, Holroyd KA. Behavioral migraine management modifies behavioral and cognitive coping in people with migraine. Headache 2014;54(9):1470-1483.
Cady RK, Goldstein J, Nett R, et al. A double blind placebo controlled pilot study of sublingual feverfew and ginger (LipiGesic M) in the treatment of migraine. Headache 2011;51(7):1078-1086.
Oelkers-Ax R, L eins A, Parzer P, et al. Butterbur root extract and music therapy in the prevention of childhood migraine: and explorative study. Eur J Pain 2008;12(3):301-313.
Brujin J, Duivenvoorden H, Passchier J, et al. Medium-dose riboflavin as a prophylactic agent in children with migraine: a preliminary placebo controlled randomized, double-blind, cross-over trial. Cephalalgia 2010;30(12):1426-1434.
Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia 2013;33(9):665-671.
Orr SL, Ventkateswaran S. Nutraceuticals in the prophylaxis of pediatric migraine: evidence-based review and recommendations. Cephalalgia 2014:34(8):568-683
Werder DS, Sargent JD. A study of childhodd headache using biofeedback as a treatment alternative. Headache 1984;24(3):122-126.
Arruda MA, Guidetti V, Galli F, et al. Primary headaches in childhood—a population-based study. Cephalalgia 2010;30(9):1056-1064.
Wojaczynska-Stanek K, Koprowski R, Wrobel Z, Gola M. Headache in children’s drawings. J. Child Neurol 2008;23(2):184-191.
Lewis D, Ashwal S, Hershey A, et al. Practice parameter: pharmacological treatment of migraine headache in children and adolescent: report of the American Academy of Neurology Quality Standards Subcommittee and the Practice Committee of the Child Neurology Society. Neurology 2004;63(12):2215-2224.
Lewis DW, Ashwal S, Dahl G, et al. Practice parameter: evaluation of children and adolescents with recurrent headaches: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology 2002;59(4):490-498.
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