hunting ton disease and other choreas

8/9/2019 Hunting Ton Disease and Other Choreas

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Box1Causes of Chorea

Genetic causes

Huntington disease

Huntington disease-like illnesses

Neuroacanthocytosis

McLeod syndrome

Wilson disease

Benign hereditary chorea (BHC)

Spinocerebellar atrophy type 2



Dentatorubropallidoluysian degeneration

Ataxia-telangiectasia

Ataxia associated with oculomotor apraxia

Neuroferritinopathy

Pantothenate kinase associated degeneration

Leigh disease and other mitochondriopathies

Lesch-Nyhan disease

Immunologic

Sydenham chorea (SC) and variants (chorea gravidarum and contraceptive-induced chorea)

Systemic lupus erythematosus

Antiphospholipid antibody syndrome

Paraneoplastic syndromes

Acute disseminated encephalomyelopathy

Celiac disease

Drug-related

Amantadine

Amphetamine

Anticonvulsants

Carbon monoxide

Central nervous system (CNS) stimulants (methylphenidate, pemoline, cyproheptadine)

Cocaine

Dopamine agonists

Dopamine-receptor blockers

Ethanol

Levodopa

Levofloxacin

Lithium

Sympathomimetics

Theophylline

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of medium spiny neurons and layers III, IV, and V of the cortex with loss of largeneurons, and is characterized by the presence of intranuclear inclusion bodiesconsisting of amyl oid-li ke fibrils that contain mutant huntingtin, ubiquitin, synuclein,and other proteins. 3537

Management

To d a te there is no effective treatment to modify the relentlessly progressive course of HD. 38 Symptomatic treatment of chorea is needed if it causes functional disability orsocial embarrassment. One principle that generally guides the choice of antichoreicagents is their ability to powerfully block D2 receptors. With the exception of amanta-dine (see later discussion), all drugs effective in providing symptomatic improvementof chorea have high affinity toward this family of dopamine receptors, which explainswhy atypical agents, such as quetiapine and clozapine, often used to treat psychosisin patients with movement disorders, are usually ineffective in controlling chorea.

Atypical antipsychotics such as olanzapine, quetiapine or risperidone may sufficientlyand at least transiently reduce choreic movements but are generally less potent thantypical neuroleptics. If the neuroleptics are effective in reducing chorea, they are oftenassociated with unacceptable side effects such as sedation, acute dystonic reaction,tardive dyskinesia, and parkinsonism. Parkinsonism can be a problem in patients withHD; with progression of the illness there is a tendency for development of rigidity anddystonia. 39 Mild to moderate chorea in HD may also respond to glutamate antagonistssuch as amantadine. 40,41 Recent controlled data have confirmed that tetrabenazine,a presynaptic dopamine depletory, is efficacious in controlling chorea in HD. 42,43

This agent has now been approved by the Food and Drug Administration for symp-

tomatic control of chorea in HD. In a recent open-label study, aripiprazole was foundto have efficacy comparable to tetrabenazine in the control of chorea in HD. Furtherstudies are required to confirm this observation. 44 Levodopa may be required inpatients with Huntington disease with advanced illness and severe rigidity. 38 Atypicalneuroleptics may also be useful in the mana gem ent of emotional irritability, aggres-siveness, and other forms of erratic behavior. 17,45 Depression may respond to classicantidepressants such as selective serotonin reuptake inhibitors (SSRIs) or antimuscar-inic drugs, or to newer antidepressants such as mirtazapine, reboxatine, or venlafaxin,but there are no formal trial data to assess the relative efficacy and safety of theseagents in HD. 17 Occasional reports have claimed mild beneficial effects of treatmentwith cholinesterase inhibitor to reduce cognitive dysfunction in HD, but there are noadequate studies to support their use. 46,47 Surgery to treat chorea is rarely needed.Pallidotomy and pallidal stimulation have also been used in a few patients withHD. 48,49 Several patients with Huntington disease have been treated with fetal cellimplantation in the striatum. Despite a few positive reports, in most cases no improve-ment has been observed. Autopsy studies have shown that the grafts survive but donot integrate with host striatum. 50,51

OTHER GENETIC CHOREAS

Approximately 3% of patients with an HD phenotype test negative for this condition.Cohort studies have established that, although most phenocopy patients remain undi-agnosed, in those patients in whom a genetic diagnosis is reached the commonestcauses are neuroacanthocytosis, SCA17, Huntington disease-like syndrome 2, familialprion disease, and Friedreich ataxia. Other causes of HD phenocopies includeHunting-ton disease-like syndrome 1, Huntington disease-like syndrome 3, other spinocerebel-lar ataxias, dentatorubral-pallidoluysian atrophy, and iron accumulation disorders. 52,53

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Neuroacanthocytosis causes chorea combined with dystonia (especially a self-mutilating oro-mandibullingual dyskinesia), tics, parkinso nism, dementia, seizures,and head of caudate atrophy on neuroimaging studies. 54 Several conditions cancause the combination of chorea and acanthocytosis: autosomal recessive chorea-acanthocytosis, X-linked McLeod syndrome, Huntington disease-like 2, pa nthotenatekinaseassociated neurodegeneration, and Bassen-Kornzweig disease. 55 A study of several patients with McLeod syndrome, including the subject of the original report,demonstrated that their phenotype is similar to the clinical features of patients withHD, with the myopathic findings overtaken by chorea and dementia. 56

Another genetic cause of chorea that has received increased attention is benignhereditary chorea (BHC). Not long ago, the existence of this condition was ques-tioned. 57 Now it is well established as an autosomal dominant illness, with a mutationin the TITF-1 gene, which codes for a transcription factor essential for the organogen-esis of the lungs, thyroid, and basal ganglia. 58 The clinical picture of these patients ischaracterized by a variable combination of chorea, mental retardation, congenitalhypothyroidism, and chronic lung disease, hence the term brain-thyroid-lungsyndrome, proposed for this disease. 59,60 The movement disorder of these patientsis differentiated from the hyperkinesia seen in myoclonus dystonia by its continuousnature; in myoclonus dystonia the movements are triggered by motion. 61 New studieswith clinical-genetic correlation have helped to demonstrate that the clinical spectrumof BHC is wider than previously believed, and includes psychosis and shortstature. 62,63 The phenotype of BHC and hypothyroidism has been shown to be causedby a novel NKX2.1 mutation. 64 Moreover, a new locus on 8q21.3 q23.3 is associatedwith adult-onset, pure, slowly progressive chorea. 65

The list of genetic causes of chorea is long and growing.1

It is beyond the scope of this article to review all of them. Recently, however, there has been interest in a fewadditional conditions in which progress has been made. For example, paroxysmalnonkinesigenic dyskinesia, Mount-Reback Syndrome, is now known to be causedby m utati ons of myofibrillogenesis regulator 1 (MR-1) gene mutations, located on2q35. 66,67 Patients with this condition develop episodes of chorea or other movementdisorde rs unrelated to exercise but often in association with the use of nicotine andalcohol. 68 A new study showed that there is a defect in the MR-1 mitochondrial target-ing sequence. 69 Other paroxysmal dyskinesias in which chorea is part of the clinicalpicture map to genes on 16p12-q12 and 10q22. 70

There are no controlled studies of treatment of other genetic causes of chorea.Overall, the principles discussed for HD apply to these conditions. However, thereare peculiarities in some cases. A substantial proportion of patients with chorea-acan-thocytosis present with a self-mutilating tongue-biting dystonia. This dyskinesia canbe disabling, preventing these patients from eating properly and resulting in severeinjuries. In the authors experience, injections of botulinum toxin in the genioglossusmuscle can provide substantial and lasting relief. Another clinical feature common inthis condition is seizure disorder, requiring the use of antiepileptic drugs. The kinesi-genic paroxysmal dyskinesias are sensitive to low doses of antepileptic drugs,whereas Mount-Reback syndrome responds to benzodiazepines. 68,71 Finally, there

is one report suggesting that levodopa can be effective in the treatment of BHC. 72

SYDENHAM CHOREA

SC, the most common form of autoimmune chorea worldwide, is a major feature of acute rheumatic fever (ARF), a nonsuppurative complication of group A b -hemolyticStreptococcus infection. Despite the decline of ARF, it remains the most common

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cause of acute chorea in children in the United States and a major public healthproblem in developing areas of the world. Clinically, it is characterized by a combina-tion of c ho rea, other movement disorders, behavioral abnormalities, and cognitivechanges. 1,2

Clinical FeaturesThe usual age at onset of SC is 8 to 9 years, but there are reports of patients whodeveloped chorea during the third decade of life. In most series, there is a femalepreponderance. 73 Typically, patients develop this disease 4 to 8 weeks after anepisode of group A b -hemolytic streptococcal pharyngitis. It does not occur afterstreptococcal infection of the skin. The chorea spreads rapidly and becomes general-ized, but hemichorea persists in 20% of patients. 73,74 Patients display motor impersis-tence, particularly noticeable during tongue protrusion and ocular fixation. The muscletone is usually decreased; in severe and rare cases (8% of all patients seen at the

Movement Disorders of the Federal University of Minas Gerais, Brazil), this is sopronounced that the patient may become bedridden (chorea paralytica).Patients often display other neurologic and nonneurologic symptoms and signs.

There are reports that tics are a common occurrence in SC. However, it is virtuallyimpossible to distinguish simple tics from fragments of chorea. Even vocal tics, foundin 70% or more of patients with SC in one study, are not a simple diagnosis in patientswith hyperkinesias. 75 In a cohort of 108 patients with SC who were carefully followedup at our unit, vocalizations were identified in just 8% of subjects. The term tic wasavoided because there was no premonitory sign or complex sound and, conversely,the vocalizations were associated with severe cranial chorea. These findings suggest

that involuntary sounds present in a few patients with SC result from choreic contrac-tions of the upper respiratory tract muscles rather than true tics. 76,77 There is evidencethat many patients with active chorea have hypometric saccades, and a few also showoculogyric crisis. Dysarthria and impairment of verbal fluency are common. In a casecontrol study of patients, a pattern of decreased verbal fluency was described thatreflected reduced phonetic, but not semantic, output. 78 This result is consistentwith dysfunction of the dorsolateral prefrontal basal ganglia circuit. Studying adultswith SC, the authors have extended this finding, showing that many functions depen-dent on the prefrontal area are impaired in these patients. The conclusion of this studyis that SC should be included among the causes of dysexecutive syndrome. 79 The

prosody is also affected in SC. In an investigation of 20 patients with SC, decreasedvocal tessitura and increased duration of the spee ch were shown. 80,81 These findingsare similar to those observed in Parkinson disease. 82 In a recent survey of 100 patientswith rheumatic fever, half of whom had chorea, migraine was f ound to be morefrequent in SC (21.8%) than in normal controls (8.1% , P 5 .02). 83 This is similar towhat has been described in Tourette syndrome (TS). 84 In the older literature, thereare also references to papilledema, central retinal artery occlusion, and seizures ina few patients with Sydenham chorea.

Attention has also been drawn to behavioral abnormalities. Swedo and colleagues 85

found obsessive-compulsive behavior in 5 of 13 SC patients, 3 of whom met criteria forobsessive-compulsive disorder, whereas no patient of the rheumatic fever grouppresented with obsessive-compulsive behavior. In another study of 30 patients withSC, Asbahr and colleagues 86 demonstrated that 70% of the subjects presentedwith obsessions and compulsions, whereas 16.7% of them met criteria for obses-sive-compulsive disorder. None of the 20 patients with ARF without chorea hadobsessions or compulsions. These results were roughly replicated by a more recentstudy in which patients with ARF without chorea were found to have more obsessions

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and compulsions than healthy controls. 87 This study also tackled the issue of hyper-activity and attention deficit disorder in SC and found that 45% of their 22 patientsmet criteria for this condition. Recently, Maia and colleagues 88 investigated behavioralabnormalities in 50 healthy subjects, 50 patients with rheumatic fever without chorea,and 56 patients with Sydenham chorea. The investigators found that obsessive-compulsive behavior, obsessive-compulsive disorder, and attention deficit and hyper-activity disorder were more frequent in the SC group (19%, 23.2%, 30.4%) than in thehealthy controls (11%, 4%, 8%) or in the patients with ARF without chorea (14%, 6%,8%). In this study, the investigators demonstrated that obsessive-compulsivebehavior displays little degree of interference in the performance of the activities of daily living. Another study compared the phenomenology of obsessions and compul-sions of patients with SC with subjects diagnosed with tic disorders. The investigatorsdemonstrated that the symptoms observed among the SC patients were different fromthose reported by patients with tic disorders but were similar to those previously notedamong samples of pediatric patients with primary obsessive-compulsive disorder. 89

A recent investigation comparing healthy controls with patients with rheumatic fevershowed that obsessive-compulsive behavior is more commonly seen in patientswith Sydenham chorea with relatives who also have obsessions and compulsions. 90

This study makes clear that there is interplay between genetic factors and the environ-ment in the development of behavioral problems in SC. The authors recently reportedthat, although rare, SC may induce psychosis or trichotillomania during the acutephase of the illness. 91,92

An investigation demonstrated that the peripheral nervous system is not targeted inSydenham chorea. 93 SC is a major manifestation of rheumatic fever. Sixty percent to

80% of patients display cardiac involvement, particularly mitral valve dysfunction, inSC, whereas the association with arthritis is less common, seen in 3 0% of patients;however, in approximately 20% of patients, chorea is the sole finding. 73,94 A prospec-tive follow-up of patients with SC with and without cardiac involvement in the firstepisode of chorea suggests that the heart remains spared in those without lesion atthe onset of the rheumatic fever. 95

The current diagnostic criteria of SC are a modification of the Jones criteria: choreawith acute or subacute onset and lack of clinical and laboratory evidence of analternative cause are mandatory findings. The diagnosis is further supported by thepresence of additional major or minor manifestations of ARF. 78,96,97 The first validated

scale to rate SC was published recently. The Universidade Federal de Minas GeraisSydenham Chorea Rating Scale was designed to provide a detailed quantitativedescription of the performance of the activities of daily living, behavioral abnormalities,and motor function of patients with SC. It comprises 27 items, and each is scored from0 (no symptom or sign) to 4 (severe disability or finding). 98

Etiology and Pathogenesis

Taranta and Stollerman established the casual relationship between infection withgroup A b -hemolytic streptococci and the occurrence of SC. 99 Based on the assump-tion of molecular mimicry between streptococcal and central nervous systemantigens, it has been proposed that the bacterial infection in genetically predisposedsubjects leads to formation of cross-reactive antibodies that disrupt the basal gangliafunction. Several studies have demonstrated the presence of such circulatingantibodies in 50% to 90% of patients with Sydenham chorea. 100,101 A specific epitopeof streptococcal M proteins that cross-reacts with basal ganglia has been identi-fied. 102 In one study, all patients with active SC had antibasal ganglia antibodiesdemonstrated by ELISA and Western blot. In subjects with persistent SC (duration

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of disease greater than 2 years despite best medical treatment), positivity was about60%. 103 Rece ntly, neuronal tubulin was found to be the target of antineuronalantibodies. 104 The biologic value of the antibasal ganglia antibodies remains to bedetermined. One study sugge sts that they may interfere with neuronal function,however. Kirvan and colleagues 105 demonstrated that IgM of 1 patient with Sydenhamchorea induced expression of calcium-dependent calmodulin in a culture of neuro-blastoma cells. Our finding that there is a linear correlation between the increase inintracellular calcium levels in PC12 cells and antibasal ganglia antibody titer in theserum from SC patients further strengthens the hypothesis that these antibodieshave a pathogenic value. 106

Although some investigations suggest that susceptibility to rheumatic chorea islinked to human leukocyte antigen-linked antigen expression, 107 some studies havefailed to identify any relationship between SC and human leukocyte antigen class Iand II alleles. 108 However, an investigation has shown that there is an associationbetween HLA-DRB1*07 and recurrent streptococcal pharyngitis and rheumatic heartdisease. 109 The genetic marker for ARF and related conditions would be the B-cellalloantigen D8/17. 110 Despite repeated reports of the group who developed the assayclaiming its high specificity and sensitivity, 111,112 findings of other investigatorssuggest that the D8/17 marker lacks specificity and sensitivity. 1 Another suggestedgenetic risk factor for development of ARF but not SC are polymorphisms within thepromoter region of the tumor necrosis factor- a gene. 113

Because of the difficulties with the molecular mimicry hypothesis in accounting forthe pathogenesis of SC, some studies have addressed the role of immune cellularmechanisms in this condition. Investigating sera and cerebrospinal fluid (CSF)

samples from patients of the Mov ement Disorders of the Federal University of MinasGerais, Church and colleagues 114 found elevation of cytokines that take part in the Th2(antibody-mediated) response, interleukins 4 (IL-4) and 10 (IL-10), in the serum of patients with acute SC in comparison with persistent SC. They also described inter-leukin 4 in the CSF of 31% of patients with acute SC, whereas just interleukin 4 wasraised in the CSF of patients with persistent SC. The investigators concluded thatSC is characterized by a Th2 response. However, as they have found elevated levelsof interleukin 12 in patients with acute SC and, more recently, Teixeira and colleaguesdescribed an increased concentration of chemokines CXCL9 and CXCL10 in theserum of patients with acute SC, 115 it can be concluded that Th1 (cell-mediated)

mechanisms may also be involved in the pathogenesis of this disorder.Currently, the evidence suggests that the pathogenesis of SC is related to circu-

lating cross-reactive antibodies. Streptococcus-induced antibodies have been shownto be associated with a form of acute disseminated encephalomyelitis characterizedby a high frequency of dystonia and other movement disorders as well as basalganglia lesions on neuroimaging. 116 Antineural and antinuclear antibodies have alsobeen found in patients with TS but their relationship with prior streptococcus infectionremains equivocal. 117

Management

In the past, physicians emphasized the need for bed rest for the treatment of SC.Currently there is no place for this measure. Because SC results from autoimmune,and not direct bacterial attack against the CNS, quarantine to prevent contaminationof others is usually unnecessary. 118

The first aim of the treatment of SC is to provide control of the chorea and behavioralproblems often associated with this condition. Regardless of the choice of agent forsymptomatic control, the physician should attempt a gradual decrease in the dosage

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of the medication (25% reduction every 2 weeks) after the patient remains free of thesymptom for at least 1 month. In some patients symptoms are so mild that they do notcause meaningful disability. In these cases, it is possible to a void a ny pharmacologicintervention, because spontaneous remission of SC is the rule. 97,119 The second aim isprophylaxis of new bouts of ARF. Although it remains un pro ved whether prophylaxis of streptococcus infection prevents recurrences of SC, 120 clearly it decreases thedevelopment of new cardiac lesions, which are the source of the most importantdisability in rheumatic fever.

There are no controlled studies of symptomatic treatment of SC and all the recom-mendations mentioned are off-label use of the cited drugs. 118 The first choice isvalproic acid at an initial dosage of 250 mg/d that is increased over a 2-week periodto 250 mg 3 times a day. If the response is not satisfactory, dosage can be increasedgradually up to 1500 mg/d. As this drug has a slow onset of action, a period of 2 weeksshould elapse before concluding that the regimen is ineffective. Valproic acid is usuallywell tolerated although some patients may develop dyspepsia and diarrhea at thebeginning of the treatment. Chronic exposure may be associated with action tremorof the hands and, more rarely, liver toxicity. An open-label study demonstrated thatcarbamazepine (15 mg/kg/d) is as effective as valproic acid (2025 mg/kg/d) to induceremission of chorea. 121 If the patient fails to respond to valproic acid, or, as first linetreatment in patients who present with chorea paralytica, the next option is toprescribe neuroleptics. Risperidone, a potent dopamine D2 receptor blocker, isusually effective in controlling the chorea. The initial regimen is usually 1 mg twicea day. If, 2 weeks later, the chorea is still troublesome, the dosage can be increasedto 2 mg twice a day. Haloperidol and pimozide are also occasionally used in the

management of chorea in SC. However, they are less well tolerated than risperidone.Dopamine D2 receptor blockers must be used with great caution in patients with SC. After observation of development of parkinsonism, dystonia, or both in patientstreated with neuroleptics, we performed a casecontrol study comparing theresponse to these drugs in patients with SC and TS. Five percent of 100 patientswith chorea developed extrapyramidal complications, whereas these findings w erenot seen among patients with tics matched for age and dosage of neuroleptics. 121

Other potential side effects of these agents are sedation, depression, and tardivedyskinesia. There are no published guidelines concerning the discontinuation of anti-choreic agents. Our policy is to attempt a gradual decrease of the dosage (25% reduc-

tion every 2 weeks) after the patient remains at least free of chorea for 1 month. Finally,the most important measure in the treatment of patients with SC is secondaryprophylaxis.

Because of the presumably autoimmune origin of SC, there have been attempts totreat patients with rheumatic chorea with corticosteroids. However, this is a controver-sial area. Despite mentions of effectiveness of prednisone in suppressing chorea, thisdrug is only used when there is associated severe carditis. We recently reported thatmethylprednisolone 25 mg/kg/d in children and 1 g/d in adults for 5 days followed byprednisone 1 mg/kg/d is an effective and well-tolerated treatment regimen for patientswith SC refractory to conventional treatment with antichoreic drugs and peni-

cillin. 98,122 At least one other group has replicated our findings of a good responseto steroids in selected patients with SC. 123 In one of the few randomized controlledtrials in SC, the investigators compared oral prednisone (2 mg/kg/d) and placebo ina double-blind fashion. Simultaneous use of haloperidol was allowed. They concludedthat steroid acc ele rates the recovery but the rate of remission and recurrence is similarin both groups. 124 However, this study has some limitations: haloperidol use was notcontrolled in both groups; it remains uncertain whether the development of side

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effects such as weight gain and moon face in the steroid group could have potentiallycompromised the blinding of the study (this is of particular concern considering thehigh dosage of prednisone); the investigators used a nonvalidated scale to rate theseverity of chorea. The current recommendation is to reserve steroids for patientswith persistent disabling chorea refractory to antichoreic agents or those who developunacceptable side effects with other agents. Finally, one open, controlled study of a small number of patients reported that plasma exchange or intravenous immuno-globulin are as effective as oral prednisone to control the severity of chorea inSC. 125 Surprisingly, the investigators report the lack of side effects in all groups.Because of the lack of additional studies to confirm the safety and effectiveness of these treatments, their high cost and existence of alternative efficacious therapeuticoptions, plasmapheresis and immunoglobulin are presently considered as investiga-tional, and do not have a place in routine medical practice.

OTHER AUTOIMMUNE CHOREAS

Other immunologic causes of chorea are systemic lupus erythematosus (SLE), primaryantiphospholipid antibody syndrome (PAPS), vasculitis, and paraneoplasticsyndromes. SLE or PAPS are classically described as the prototypes of autoimmunechoreas. 126 However, several reports show that chorea is seen in no more than 1% to2% of large series of patients with these conditions. 127,128 Autoimmune chorea hasrarely been reported in the context of paraneoplastic syndromes associated withCV2/CRMP5 antibodies in patients with small-cell lung carcinoma or malignantthymoma. 129 As these disorders are rare, chorea caused by them is uncommon.Chorea associated with SLE or PAPS has been treated with immunosuppressivemeasures, especially intravenous methylprednisolone following a dosage regimenas described for SC, and intravenous immunoglobulin. 130 As it is accepted thatneurologic complications, including chorea, in PAPS are related to ischemic events,antiplatelet agents and even anticoagulants are often prescribed to treat chorea inthis condition. 131 However, these recommendations are based on reports of open-label studies involving small numbers of patients and the clinical experience of thephysicians. 1

VASCULAR CHOREAS

A study in a tertiary referral center showed that cerebrovascular disease was the mostcommon cause of nongenetic chorea, accounting for 21 out of 42 cases. 3 Conversely,chorea is an unusual complication of acute vascular lesions, seen in less than 1% of patients with acute stroke. Vascular hemichorea, or hemiballism, is usually related toischemic or hemorrhagic lesions of the basal ganglia and adjacent white matter in theterritory of the middle or the posterior cerebral artery. In contrast to the classictextbook concepts of he mib allism, most patients with vascular chorea have lesionsoutside the subthalamus. 132 Although spontaneous remission is the rule, treatmentwith antichoreic drugs such as neuroleptics or dopamine depletors may be necessaryin the acute phase. In a few patients with vascular chorea, the movement disorder maypersist. These patients can be treated effectively with stereotactic surgery such asthalamotomy or posteroventral pallidotomy. 133,134

An uncommon cause of chorea is Moyamoya disease, an intracranial vasculopathythat presents with an ischemic lesion or, less commonly, hemorrhagic stroke of thebasal ganglia. 135 Another rare form of vascular chorea is postpump chorea,a complication of extracorporeal circulation. The pathogenesis of this movementdisorder is believed to be related to vascular insult of the basal ganglia during the

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surgical procedure. The current evidence supports the notion that the long-term prog-nosis of PC is poor. In 1 series of 8 patients, for example, 5 subjects had persistentchorea and 1 of them died. In another study, there was a clear distinction betweenthose 8 patients with onset at earlier age (median 4.3 months), all of whom recoveredfully, and 11 others, who were older at onset (median age 16.8 months). Amon g thelatter, 4 died and only 1 of the survivors made a complete neurologic recovery. 136

SUMMARY

Huntington disease (HD), an autosomal dominant degenerative disease, is the mostcommon genetic cause of chorea. HD, a relentlessly progressive illness, is character-ized by a combination of movement disorder, cognitive decline, and behavioral abnor-malities. There is no treatment capable of stopping its progression. The most commonconditions that mimic HD are neuroacanthocytosis, SCA17, Huntington disease-likesyndrome 2, familial prion disease, and Friedreich ataxia. Sydenham chorea (SC) isthe most common cause of chorea in children worldwide. Patients with SC presentwith a variable combination of chorea, obsessive-compulsive behavior, otherbehavioral abnormalities, and carditis. Management of SC involves prophylaxis of Streptococcus infections with penicillin and antichoreic agents. Stroke is the mostcommon sporadic cause of chorea in adults. Vascular chorea, usually a complicationof diabetes mellitus, is often a self-limited condition.

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