eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases

Chorea in Adults

Author: Stephanie M Vertrees, MD, Staff Physician, Section of Internal Medicine, Department of Neurology, Dartmouth-Hitchcock Medical Center
Coauthor(s): Stephen A Berman, MD, PhD, Professor, Department of Internal Medicine, Section of Neurology, Dartmouth Medical School; Chief, Neurology Service, White River Junction Veterans Medical Center
Contributor Information and Disclosures

Updated: May 7, 2009

Introduction

Background

"Chorea" is a borrowed Latin word that derives from the Greek khoreia, a choral dance. The basic Greek word for dance (written with the Roman alphabet) is khoros.1,2

The ad hoc Committee on Classification of the World Federation of Neurology has defined chorea as "a state of excessive, spontaneous movements, irregularly timed, non-repetitive, randomly distributed and abrupt in character. These movements may vary in severity from restlessness with mild intermittent exaggeration of gesture and expression, fidgeting movements of the hands, unstable dance-like gait to a continuous flow of disabling, violent movements."3

Patients with chorea exhibit motor impersistence (ie, they cannot maintain a sustained posture). When attempting to grip an object, they alternately squeeze and release ("milkmaid's grip"). When they attempt to protrude the tongue, the tongue often pops in and out ("harlequin's tongue"). Patients often drop objects involuntarily. Also common are attempts by patients to mask the chorea by voluntarily augmenting the choreiform movements with semipurposeful movements.1

Chorea involves both proximal and distal muscles. In most patients, normal tone is noted, but, in some instances, hypotonia is present. In a busy movement disorder center, levodopa-induced chorea is the most common movement disorder, followed by Huntington disease (HD).1

Any discussion of chorea must also address the related terms athetosis, choreoathetosis, and ballism (also known as ballismus).

The term athetosis comes from the Greek word athetos (not fixed).1,2 It is a slow form of chorea. Because of the slowness, the movements have a writhing (ie, squirming, twisting, or snakelike) appearance. Choreoathetosis is essentially an intermediate form (ie, a bit more rapid than the usual athetosis, slower than the usual chorea, or a mingling of chorea and athetosis within the same patient at different times or in different limbs). Given that the only difference between chorea, choreoathetosis, and athetosis is the speed of movement, some neurologists argue that the term athetosis is unnecessary and even confusing. They argue a simpler nomenclature would delineate fast, intermediate, and slow chorea. While the authors of this article understand the basis of that argument, they also believe that in some cases, the writhing movements are extremely prominent, even apart from the speed of the movement. Thus, the authors of this article advocate retaining this descriptive term.

Ballism or ballismus is considered a very severe form of chorea in which the movements have a violent, flinging quality. In Greek, ballismos means "a jumping about or dancing."2 Ballism has been defined as "continuous, violent, coordinated involuntary activity involving the axial and proximal appendicular musculature such that the limbs are flung about." This movement disorder most often involves only one side of the body (ie, hemiballism or hemiballismus). Occasionally, bilateral movements occur (ie, biballism or paraballism). Many patients with hemiballism have choreiform movements and vice versa, and hemiballism often evolves into hemichorea. Currently, ballism should be viewed as a severe form of chorea.1,4,5,6,7,8

Pathophysiology

A simple model of basal ganglia function states that dopaminergic and GABAergic impulses from the substantia nigra and motor cortex, respectively, are funneled through the pallidum into the motor thalamus and motor cortex. These impulses are modulated in the striatum via two segregated, parallel, direct and indirect loops through the medial pallidum and lateral pallidum/subthalamic nucleus. Subthalamic nucleus activity drives the medial pallidum to inhibit cortex-mediated impulses, thereby inducing parkinsonism. Absent subthalamic nucleus inhibition enhances motor activity through the motor thalamus, resulting in abnormal involuntary movements such as dystonia, chorea, and tics. A classic example of loss of subthalamic inhibitory drive is ballism.1

The most well-studied choreatic syndrome is Huntington chorea; therefore, the pathophysiology of HD as it applies to chorea is the focus of the discussion that follows.

Huntington disease is caused by an expanded CAG trinucleotide repeat in the gene that encodes the protein huntingtin. Mutant huntingtin is thought to cause neuronal degeneration through transcription dysregulation as well as mitochondrial impairment.9,10,11,12

Dopaminergic mechanism

In Huntington chorea, the content of striatal dopamine is normal, indicating that the major pathological alterations lay in the surviving — but diseased — medium-sized, spiny, striatal dopaminergic neurons. Pharmacologic agents that either deplete dopamine (eg, reserpine and tetrabenazine) or block dopamine receptors (eg, neuroleptic medications) improve chorea, which gives further support to this observation. Given that drugs that decrease the striatal content of dopamine improve chorea, increasing the amount of dopamine worsens chorea, such as in the levodopa-induced chorea seen in persons with Parkinson disease (PD).13,14

Cholinergic mechanism15

The concept that a critical striatal balance between acetylcholine (Ach) and dopamine is essential for normal striatal function received its greatest acceptance in the understanding of PD. In the early days of PD therapy, anticholinergic medications were used frequently, especially when tremor was the predominant symptom. Other PD symptoms, such as bradykinesia and rigidity, often improved as well.

The development of chorea in patients treated with anticholinergic medications, such as trihexyphenidyl, is a common clinical observation. Furthermore, the intravenous administration of physostigmine (a centrally acting anticholinesterase) can temporarily reduce chorea. The same treatment can also promptly overcome anticholinergic-induced chorea.

Patients with HD have a patchy reduction of choline acetyltransferase in the basal ganglia. This enzyme catalyzes the synthesis of ACh. A marked reduction of muscarinic cholinergic receptor sites has also been reported. These two observations could explain the variability of patients' response to physostigmine and the limited efficacy of Ach precursors such as choline and lecithin.

Serotonergic mechanism

Fluctuations in striatal serotonin may play a role in the genesis of many abnormal movements. Selective serotonin reuptake inhibitors, such as fluoxetine, may induce or aggravate parkinsonism, akinesia, myoclonus, or tremor. The role of serotonin (5-hydroxytryptamine [5-HT]) in choreiform movements is less clear since the striatum has a relatively high concentration of serotonin. Pharmacologic attempts to either stimulate or inhibit serotonin receptors in persons with Huntington chorea have shown no effect, indicating that serotonin's contribution to the pathogenesis of chorea is limited.

GABAergic mechanism

The most consistent biochemical lesion in patients with Huntington chorea appears to be a loss of neurons in the basal ganglia that synthesize and contain GABA.16 The significance of this remains unknown. A variety of pharmacologic techniques have been attempted to increase CNS GABA levels. Valproic acid, which acts in part via a GABAergic mechanism, has, in a limited number of uncontrolled cases, ameliorated not only the agitation sometimes seen in persons with HD but also the movement problem.17 However, no systematic studies have been conducted on the use of GABAergic agents to treat HD.

Substance P and somatostatin

Substance P levels have been shown to be markedly lower in persons with Huntington disease (HD), while somatostatin levels are higher. The significance of this remains unknown as well.

Cannabinoids

 Endocannabinoids are thought to play a role in HD. Loss of the cannabinoid CB1 receptor from the medium spiny neurons is one of the earliest neurochemical changes seen in HD. Reuptake inhibition of anandamine, an endogenous cannabinoid, has been shown to alleviate motor symptoms in animal models of HD and other neurodegenerative disorders such as PD and MS.18,19,16

Ballism

This movement disorder usually involves only one side of the body (ie, hemiballism). Hemiballism is usually attributed to lesions of the contralateral subthalamic nucleus, although infarction in the caudate, striatum, lenticular nucleus, or thalamus has also been associated with hemiballism.1,4

Lesions of the subthalamic nucleus can cause contralateral hemiballism-hemichorea by reducing the normal excitatory drive from the subthalamic nucleus to the internal segment of the globus pallidus. This reduces the inhibitory output of the globus pallidus on the thalamus, and this disinhibition gives rise to excessive excitatory drive to the cortex, which is expressed as contralateral hyperkinetic movements. Confusingly, however, this disorder often appears in the absence of a lesion in the subthalamic nucleus.1,20

Klawans21,22 suggested that increased dopaminergic transmission might play a role in the pathophysiology of this disorder. This hypothesis is supported by the observation that dopamine-receptor blockers and catecholamine-depleting agents often improve hemiballism. While hemiballism and hemichorea are distinguishable on the basis of the type and distribution of movements, they represent two different symptoms on a spectrum of the same disease process. Why one patient with basal ganglia dysfunction develops hemiballism and another with similar pathologic changes develops hemichorea is not understood. On the cellular and molecular level, ballism can be caused by multiple pathologies including ischemia, infection, demyelination, and tumor.5,6,23,24,25,26,7,8

Frequency

United States

Although no data are available regarding the incidence of chorea, the incidences of several disorders in which chorea is the main clinical feature are well known.

  • Huntington disease (HD) is an autosomal dominant, neurodegenerative disorder in which the defective gene is located on the short arm of chromosome 4. The estimated prevalence of HD in the United States is 5-10 cases per 100,000 people.1
  • Wilson disease is an autosomal recessive, multisystem disease caused by a mutation in the ATP7B gene, which resides on the long arm (q) of chromosome 13 (13q14.3). This gene codes for an ATPase, which is involved with the transport of copper. Although the gene prevalence (heterozygous carriers who inherited only 1 abnormal gene) has been estimated to be as high as 1%, the disease prevalence is only 30 cases per 1 million people.1,27,28
  • Benign hereditary chorea, a fairly rare disorder in which most of the pedigrees have clearly demonstrated dominant inheritance, has a prevalence of approximately 1 case per 500,000 people.1,29,30,31,32,33,34,35,36,37,38,39,40,41

Race

  • In 1872, George Huntington first described HD inheritance in successive generations of natives of Long Island, New York. All of the affected individuals descended from ancestors who had emigrated from East Anglia, England, to the New World in 1649. This disorder is now dispersed widely around the globe.
  • HD is best known in white populations. All cases of the disorder are probably part of the line originating in East Anglia.
  • In addition, informative genotypes were obtained from a vast family lineage carrying the gene; they are located in and around Lake Maracaibo, Venezuela.

Age

Chorea can commence at any age. In children, postpump chorea and infectious, inflammatory, and striatal lesions may account for many cases.

  • For Huntington disease (HD), the typical age at onset is in the 40s or 50s. Cases have been recognized in patients younger than 5 years, but generally no more than 10% of the cases show onset prior to age 20. Patients with early onset usually inherited the disease from their father, while patients with later onset are more likely to have inherited the gene from their mother. The relatively low rate of expression in childhood is succeeded by a virtually exponential upsweep in the rate of appearance through the 20s and 30s to reach a plateau that is sustained from the 40s to the 70s. Although 27% of cases are first recognized in patients older than 50 years, most of the cases are documented in patients younger than 60 years. Onset has been recorded as late as the eighth decade.1,42
  • Neuroacanthocytosis, perhaps the most common form of hereditary chorea, usually manifests clinically in the 30s or 40s (age range is 8-62 y). It should be differentiated from late-onset HD through careful pedigree analysis and neurogenetic testing.1,27,43
  • Senile chorea manifests gradually in middle-to-late life.
  • In general, on the basis of age at onset, benign hereditary chorea may be divided into 3 types: (1) early infancy, (2) approximately 1 year of age, and (3) late childhood or adolescence. The most common type is the second; children are usually around 1 year old when they begin to walk. Benign hereditary chorea is now known to be caused by a mutation in the TITF1 gene. Interestingly, this gene contains the code for a transcription factor essential for the organogenesis of the basal ganglia, lungs, and thyroid.30,44

Clinical

History

Patients with chorea may not initially be aware of the abnormal movements because they may be subtle. Patients can suppress the chorea temporarily and frequently camouflage some of the movements by incorporating them into semipurposeful activities (ie, parakinesia). The inability to maintain voluntary contraction (ie, motor impersistence), as is seen during manual grip (milkmaid grip) tests or tongue protrusion, is a characteristic feature of chorea and results in the dropping of objects and clumsiness. Muscle stretch reflexes are often hung-up and pendular. In severely affected patients, a peculiar dancelike gait may be noted. Depending on the underlying cause of the chorea, other motor symptoms include dysarthria, dysphagia, postural instability, ataxia, dystonia, and myoclonus. A brief discussion of the clinical manifestations of the most common choreatic diseases is presented.

  • Huntington disease1,42
    • Penetrance of HD is 100%. Expression is highly variable, both with respect to clinical manifestations and age of onset. When the disorder emerges early, particularly in patients younger than 20 years, it is most likely to run a rapid course with grave disability due to cognitive decline.
    • The Westphal variant, a rigid dystonic disorder, may be accompanied by seizures and even myoclonus. It is encountered principally among those with childhood onset. In contrast, when the disorder appears late in life, the cardinal manifestation is chorea.
    • The insidious onset of clumsiness and adventitious movements may be wrongly attributed to simple nervousness. Although chorea and other motor disabilities are the most readily recognized manifestations of HD, they may be neither the earliest to appear nor the most disabling manifestations of the disease.
    • Psychological disturbances and personality change are the initial manifestations in greater than 50% of affected persons. Symptoms consistent with a depressive state are the most frequent psychological disturbances.
    • The duration of illness from onset to death is approximately 15 years in the case of adult HD and 8-10 years for the juvenile variant.
  • Wilson disease27,28
    • The clinical features are age dependent. In children, the disease is manifested initially by progressive dystonia, rigidity and dysarthria, and hepatic dysfunction, whereas in adults, psychiatric symptoms, tremor, and dysarthria usually predominate.
    • Because Kayser-Fleischer rings are almost always present when neurological symptoms are present, slit-lamp examination of the cornea must be performed to be certain that Wilson disease is excluded in a patient with chorea beginning in childhood or young adulthood. In patients with chorea and negative findings from a slit-lamp examination, serum copper and ceruloplasmin analysis along with a 24-hour copper urine excretion test need to be performed.
  • Neuroacanthocytosis1,43
    • Symptoms usually begin with lip and tongue biting (often causing self-injury), orolingual dystonia, motor and phonic tics, generalized chorea, parkinsonism, and seizures. Patients with neuroacanthocytosis may report an inability to feed themselves because of dystonic tongue protrusion every time they try to eat.
    • Other features include cognitive and personality changes, dysphagia, dysarthria, amyotrophy, areflexia, evidence of axonal neuropathy with absent deep ankle tendon stretch reflexes, and elevated serum creatine kinase levels without evidence of myopathy.
  • Senile chorea45,46,47
    • This clinical entity is characterized by a gradual onset of generalized and symmetric chorea with slow progression and specifically excluding mental deterioration, emotional disturbances, or family history.
    • To rule out the possibility of HD, genetic testing is recommended because family history can be inaccurate and distinguishing age-related mental changes from early features of HD in an elderly person may be difficult.
  • Sydenham chorea48,49
    • Sydenham chorea is a major manifestation of acute rheumatic fever. With the 1992 modifications of the Jones criteria, it alone is sufficient to enable the physician to make the diagnosis of the first attack of acute rheumatic fever. Sydenham chorea is considered a disease of childhood; however, it also may be seen in adults. Rheumatic chorea is characterized by muscle weakness and the presence of chorea. The patients have the milkmaid grip sign, clumsy gait, and explosive bursts of dysarthric speech. Often, harlequin tongue, which pops in and out when the patient tries to hold it out, can be prominently demonstrated.
    • Psychological symptoms are equally prominent and typically precede the appearance of even the most subtle choreiform movements. Emotional lability is the most common symptom; decreased attention span, obsessive-compulsive symptoms, and separation anxiety disorder also are seen. Symptoms can lag behind the etiologic streptococcal infection by 1-6 months. In adults, generalized poststreptococcal chorea may complicate birth control or pregnancy (chorea gravidarum).
  • Benign hereditary chorea1,29,30,31,32,33,34,35,36,37,38,39,40,41
    • This is a rare autosomal dominant genetic disorder characterized by nonprogressive choreiform movements that appear in childhood, without intellectual impairment. It is further distinguished clinically from juvenile HD by the absence of seizures, rigidity, or cerebellar features.
    • Benign hereditary chorea is caused by a mutation in the TITF1 gene. Interestingly, this gene contains the code for a transcription factor essential for the organogenesis of the basal ganglia, the lungs, and the thyroid.
    • It does not shorten the life span of affected patients, but severely affected patients can be markedly disabled by the chorea.

Physical

Because Huntington disease (HD) is the most clearly defined choreatic disease, its physical findings are described here.

  • Huntington disease1,50,42
    • HD is caused by an expansion repeat (CAG) mutation in the IT15 gene (which codes for the protein called huntingtin) on chromosome 4. Initial signs of chorea generally are flickers in the fingers and ticlike grimaces of the face. Over time, higher-amplitude dancelike movements disrupt voluntary actions of the extremities and interfere with gait. Speech becomes dysrhythmic.
    • Characteristically, the patient is hypotonic, although reflexes may be augmented and clonus may be noted.
    • Voluntary gaze is disturbed early. In particular, saccades may be irregular or of prolonged latency and may require an initial blink for their initiation.
    • Loss of optokinetic nystagmus is common after a decade of progressive disease.
    • Cognitive changes are manifested early as loss of recent memory and impaired judgment. Apraxia is also present. Ultimately, the patient becomes severely demented.
    • Neurobehavioral changes typically consist of personality changes, apathy, social withdrawal, agitation, impulsiveness, depression, mania, paranoia, delusions, hostility, hallucinations, or psychosis.
    • The Westphal variant is dominated by rigidity, bradykinesia, and dystonic postures. Generalized seizures and myoclonus may be seen. Ataxia and dementia are also present.

Causes

  • Idiopathic - Physiological chorea of infancy, buccal-oral-lingual dyskinesia, senile chorea45,46,47
  • Hereditary - HD, hereditary nonprogressive chorea (benign hereditary chorea)1,29,30,31,32,33,34,35,36,37,38,39,40,41 , benign recessively inherited choreoathetosis of early onset51 , familial inverted chorea52 , neuroacanthocytosis43 , familial remitting chorea nystagmus and cataracts53 , ataxia-telangiectasia, tuberous sclerosis54 , familial calcification of basal ganglia, pantothenate kinase associated neurodegeneration (PKAN) or pantothenate kinase 2 (PANK2) deficiency (previously termed Hallervorden-Spatz disease), Friedreich ataxia, dentatorubro-pallidoluysian atrophy55 .
  • Hereditary (metabolic) - Wilson disease27,28 , glutaric aciduria, Lesch-Nyhan disease, phenylketonuria, acute intermittent porphyria, propionic acidemia56 , abetalipoproteinemia, hypobetalipoproteinemia, lipid storage diseases
  • Other metabolic and endocrine disorders -Kernicterus, hyperthyroidism, hypoparathyroidism, hypoglycemia57 , nonketotic hyperglycemia58 , chorea gravidarum, hypomagnesemia, chronic nonfamilial hepatic encephalopathy59 , anoxic encephalopathy (including postcardiac transplantation)60 , cardiac surgery61 , postportocaval anastomosis for portal hypertension
  • Paroxysmal - Paroxysmal kinesogenic choreoathetosis, paroxysmal dystonic choreoathetosis
  • Infectious - Sydenham chorea, encephalitides62 , subacute sclerosing panencephalitis, syphilis, enteric cytopathogenic human orphan (ECHO) virus infection62 , Lyme disease, HIV infection7,63 , cerebral toxoplasmosis, Creutzfeldt-Jakob disease, subacute bacterial endocarditis
  • Drug induced - Neuroleptics, levodopa, anticholinergics, oral contraceptives, antihistamines, amphetamines, cocaine, phenytoin, tricyclics
  • Toxins - Alcohol intoxication and withdrawal, carbon monoxide64,65 , manganese, mercury
  • Vascular - Cerebrovascular disease (ischemic or hemorrhagic)66,5,24,67,68,69 , chronic subdural hematoma70 , Moyamoya disease71 , migraine/hemicrania choreatica72 , Churg-Strauss syndrome73 , polycythemia vera
  • Immunologic -Systemic lupus erythematosus, Behçet disease74 , primary antiphospholipid antibody syndrome75,76 , multiple sclerosis, postcardiac transplantation60 , postvaccination
  • Tumors - Primary, metastatic
  • Miscellaneous - Mitochondrial cytopathies, ventriculoperitoneal shunts77 , cardiac sugery61

More on Chorea in Adults

Overview: Chorea in Adults
Differential Diagnoses & Workup: Chorea in Adults
Treatment & Medication: Chorea in Adults
Follow-up: Chorea in Adults
References
[ CLOSE WINDOW ]

References

  1. Berman SA. Chorea. In: Joseph AB, Young RR, eds. Movement Disorders in Neurology and Neuropsychiatry. 2nd ed. Malden, Mass: Blackwell Science; 1999:481-94.

  2. Dorland WA, ed. Dorlands Illustrated Medical Dictionary. 30th ed. Philadelphia, Pa: WB Saunders; 2003.

  3. Barbeau A, Duvoisin RC, Gerstenbrand F, Lakke JP, Marsden CD, Stern G. Classification of extrapyramidal disorders. Proposal for an international classification and glossary of terms. J Neurol Sci. Aug 1981;51(2):311-27. [Medline].

  4. Dewey RB Jr, Jankovic J. Hemiballism-hemichorea. Clinical and pharmacologic findings in 21 patients. Arch Neurol. Aug 1989;46(8):862-7. [Medline].

  5. Fukui T, Hasegawa Y, Seriyama S, et al. Hemiballism-hemichorea induced by subcortical ischemia. Can J Neurol Sci. Nov 1993;20(4):324-8. [Medline].

  6. Glass JP, Jankovic J, Borit A. Hemiballism and metastatic brain tumor. Neurology. Feb 1984;34(2):204-7. [Medline].

  7. Sanchez-Ramos JR, Factor SA, Weiner WJ, Marquez J. Hemichorea-hemiballismus associated with acquired immune deficiency syndrome and cerebral toxoplasmosis. Mov Disord. 1989;4(3):266-73. [Medline].

  8. Vidakovic A, Dragasevic N, Kostic VS. Hemiballism: report of 25 cases. J Neurol Neurosurg Psychiatry. Aug 1994;57(8):945-9. [Medline].

  9. Chen-Plotkin AS, Sadri-Vakili G, Yohrling GJ, Braveman MW, Benn CL, Glajch KE, et al. Decreased association of the transcription factor Sp1 with genes downregulated in Huntingtons disease. Neurobiol Dis. May 2006;22(2):233-41. [Medline].

  10. Smith KM, Matson S, Matson WR, Cormier K, Del Signore SJ, Hagerty SW. Dose ranging and efficacy study of high-dose coenzyme Q10 formulations in Huntingtons disease mice. Biochim Biophys Acta. Jun 2006;1762(6):616-26. [Medline].

  11. Stack EC, Smith KM, Ryu H, Cormier K, Chen M, Hagerty SW, et al. Combination therapy using minocycline and coenzyme Q10 in R6/2 transgenic Huntingtons disease mice. Biochim Biophys Acta. Mar 2006;1762(3):373-80. [Medline].

  12. Zuccato C, Belyaev N, Conforti P, Ooi L, Tartari M, Papadimou E, et al. Widespread disruption of repressor element-1 silencing transcription factor/neuron-restrictive silencer factor occupancy at its target genes in Huntingtons disease. J Neurosci. Jun 27 2007;27(26):6972-83. [Medline].

  13. Leavitt BR, Hayden MR. Is tetrabenazine safe and effective for suppressing chorea in Huntingtons disease?. Nat Clin Pract Neurol. Oct 2006;2(10):536-7. [Medline].

  14. Savani AA, Login IS. Tetrabenazine as antichorea therapy in Huntington disease: a randomized controlled trial. Neurology. Mar 6 2007;68(10):797; author reply 797. [Medline].

  15. Gomez-Anson B, Alegret M, Munoz E, Sainz A, Monte GC, Tolosa E. Decreased frontal choline and neuropsychological performance in preclinical Huntington disease. Neurology. Mar 20 2007;68(12):906-10. [Medline].

  16. Glass M, Dragunow M, Faull RL. The pattern of neurodegeneration in Huntingtons disease: a comparative study of cannabinoid, dopamine, adenosine and GABA(A) receptor alterations in the human basal ganglia in Huntingtons disease. Neuroscience. 2000;97(3):505-19. [Medline].

  17. Saft C, Lauter T, Kraus PH, Przuntek H, Andrich JE. Dose-dependent improvement of myoclonic hyperkinesia due to Valproic acid in eight Huntingtons Disease patients: a case series. BMC Neurol. Feb 28 2006;6:11. [Medline].

  18. Curtis MA, Faull RL, Glass M. A novel population of progenitor cells expressing cannabinoid receptors in the subependymal layer of the adult normal and Huntingtons disease human brain. J Chem Neuroanat. Apr 2006;31(3):210-5. [Medline].

  19. de Lago E, Fernandez-Ruiz J, Ortega-Gutierrez S, Cabranes A, Pryce G, Baker D, et al. UCM707, an inhibitor of the anandamide uptake, behaves as a symptom control agent in models of Huntingtons disease and multiple sclerosis, but fails to delay/arrest the progression of different motor-related disorders. Eur Neuropsychopharmacol. Jan 2006;16(1):7-18. [Medline].

  20. Dubinsky RM, Greenberg M, Di Chiro G, et al. Hemiballismus: study of a case using positron emission tomography with 18fluoro-2-deoxyglucose. Mov Disord. 1989;4(4):310-9. [Medline].

  21. Klawans HL. Chorea. Can J Neurol Sci. Aug 1987;14(3 Suppl):536-40. [Medline].

  22. Evidente VG, Gwinn-Hardy K, Caviness JN, Alder CH. Risperidone is effective in severe hemichorea/hemiballismus. Mov Disord. Mar 1999;14(2):377-9. [Medline].

  23. Inzelberg R, Korczyn AD. Persistent hemiballism in Parkinsons disease. J Neurol Neurosurg Psychiatry. Aug 1994;57(8):1013-4. [Medline].

  24. Johnson WG, Fahn S. Treatment of vascular hemiballism and hemichorea. Neurology. Jul 1977;27(7):634-6. [Medline].

  25. Martinez-Martin P. Hemichorea-hemiballism in AIDS. Mov Disord. 1990;5(2):180. [Medline].

  26. Riley D, Lang AE. Hemiballism in multiple sclerosis. Mov Disord. 1988;3(1):88-94. [Medline].

  27. Jankovic J. Huntingtons disease, Wilsons disease, and neuroacanthocytosis. A Comprehensive Review of Movement Disorders for the Clinical Practitioner. 2nd Annual Course. New York, NY: Columbia University; 1992:261-78.

  28. Petrukhin K, Lutsenko S, Chernov I, et al. Characterization of the Wilson disease gene encoding a P-type copper transporting ATPase: genomic organization, alternative splicing, and structure/function predictions. Hum Mol Genet. Sep 1994;3(9):1647-56. [Medline].

  29. Bird TD, Carlson CB, Hall JG. Familial essential (benign) chorea. J Med Genet. Oct 1976;13(5):357-62. [Medline].

  30. Breedveld GJ, van Dongen JW, Danesino C, et al. Mutations in TITF-1 are associated with benign hereditary chorea. Hum Mol Genet. Apr 15 2002;11(8):971-9. [Medline].

  31. Burns J, Neuhauser G, Tomasi L. Benign hereditary non-progressive chorea of early onset. Clinical genetics of the syndrome and report of a new family. Neuropadiatrie. Nov 1976;7(4):431-8. [Medline].

  32. Chun RW, Daly RF, Mansheim BJ Jr, Wolcott GJ. Benign familial chorea with onset in childhood. JAMA. Sep 24 1973;225(13):1603-7. [Medline].

  33. Damasio H, Antunes L, Damasio AR. Familial nonprogressive involuntary movements of childhood. Ann Neurol. Jun 1977;1(6):602-3. [Medline].

  34. Haerer AF, Currier RD, Jackson JF. Hereditary nonprogressive chorea of early onset. N Engl J Med. Jun 1 1967;276(22):1220-4. [Medline].

  35. Kuwert T, Lange HW, Langen KJ, et al. Normal striatal glucose consumption in two patients with benign hereditary chorea as measured by positron emission tomography. J Neurol. Apr 1990;237(2):80-4. [Medline].

  36. MacMillan JC, Morrison PJ, Nevin NC, Shaw DJ, Harper PS, Quarrell OW, et al. Identification of an expanded CAG repeat in the Huntington's disease gene (IT15) in a family reported to have benign hereditary chorea. J Med Genet. Dec 1993;30(12):1012-3. [Medline].

  37. Rice E, Terrence C. Computerized tomography in hereditary nonprogressive chorea. Arch Neurol. Apr 1979;36(4):249-50. [Medline].

  38. Robinson RO, Thornett CE. Benign hereditary chorea--response to steroids. Dev Med Child Neurol. Dec 1985;27(6):814-6. [Medline].

  39. Suchowersky O, Hayden MR, Martin WR, et al. Cerebral metabolism of glucose in benign hereditary chorea. Mov Disord. 1986;1(1):33-44. [Medline].

  40. Wheeler PG, Weaver DD, Dobyns WB. Benign hereditary chorea. Pediatr Neurol. Sep-Oct 1993;9(5):337-40. [Medline].

  41. Yapijakis C, Kapaki E, Zournas C, Rentzos M, Loukopoulos D, Papageorgiou C. Exclusion mapping of the benign hereditary chorea gene from the Huntingtons disease locus: report of a family. Clin Genet. Mar 1995;47(3):133-8. [Medline].

  42. McKusick V. Huntington disease; HD. http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=143100. Online Mendelian Inheritance in Man. Available at http://www.ncbi.nlm.nih.gov. Accessed March 17, 2009.

  43. McKusick V. Choreoacanthocytosis; CHAC. http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=200150. Online Mendelian Inheritance in Man. Available at http://www.ncbi.nlm.nih.gov. Accessed March 17, 2009.

  44. McKusick V. Chorea, benign hereditary; BHC. http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=118700. Online Mendelian Inheritance in Man. Available at http://www.ncbi.nlm.nih.gov. Accessed March 17, 2009.

  45. Alcock, NS. A note of the pathology of senile chorea (non-hereditary). Brain. 1936;59:376-87.

  46. Friedman JH, Ambler M. A case of senile chorea. Mov Disord. 1990;5(3):251-3. [Medline].

  47. Galvez-Jimenez N, Friedman J, Lang A. A consistent MRI pattern in three cases of senile chorea. Neurology. 1995;45 (Supplement 4):A185.

  48. Giedd JN, Rapoport JL, Kruesi MJ, Parker C, Schapiro MB, Allen AJ, et al. Sydenhams chorea: magnetic resonance imaging of the basal ganglia. Neurology. Dec 1995;45(12):2199-202. [Medline].

  49. Swedo SE. Sydenhams chorea. A model for childhood autoimmune neuropsychiatric disorders. JAMA. Dec 14 1994;272(22):1788-91. [Medline].

  50. Gusella JF, MacDonald ME. Huntingtons disease: seeing the pathogenic process through a genetic lens. Trends Biochem Sci. July 2006;31(pt 9):533-40.

  51. Nutting PA, Cole BR, Schimke RN. Benign, recessively inherited choreo-athetosis of early onset. J Med Genet. Dec 1969;6(4):408-10. [Medline].

  52. Fisher M, Sargent J, Drachman D. Familial inverted choreoathetosis. Neurology. Dec 1979;29(12):1627-31. [Medline].

  53. Wheeler PG, Dobyns WB, Plager DA, Ellis FD. Familial remitting chorea, nystagmus, and cataracts. Am J Med Genet. Dec 1 1993;47(8):1215-7. [Medline].

  54. Evans BK, Jankovic J. Tuberous sclerosis and chorea. Ann Neurol. Jan 1983;13(1):106-7. [Medline].

  55. Ross CA, Margolis RL, Rosenblatt A, et al. Huntington disease and the related disorder, dentatorubral-pallidoluysian atrophy (DRPLA). Medicine (Baltimore). Sep 1997;76(5):305-38. [Medline].

  56. Sethi KD, Ray R, Roesel RA, et al. Adult-onset chorea and dementia with propionic acidemia. Neurology. Oct 1989;39(10):1343-5. [Medline].

  57. Hefter H, Mayer P, Benecke R. Persistent chorea after recurrent hypoglycemia. A case report. Eur Neurol. 1993;33(3):244-7. [Medline].

  58. Linazasoro G, Urtasun M, Poza JJ, et al. Generalized chorea induced by nonketotic hyperglycemia. Mov Disord. 1993;8(1):119-20. [Medline].

  59. Toghill PJ, Johnston AW, Smith JF. Choreoathetosis in porto-systemic encephalopathy. J Neurol Neurosurg Psychiatry. Aug 1967;30(4):358-63. [Medline].

  60. Blunt SB, Brooks DJ, Kennard C. Steroid-responsive chorea in childhood following cardiac transplantation. Mov Disord. Jan 1994;9(1):112-4. [Medline].

  61. Curless RG, Katz DA, Perryman RA, et al. Choreoathetosis after surgery for congenital heart disease. J Pediatr. May 1994;124(5 Pt 1):737-9. [Medline].

  62. Peters AC, Vielvoye GJ, Versteeg J, et al. ECHO 25 focal encephalitis and subacute hemichorea. Neurology. May 1979;29(5):676-81. [Medline].

  63. Sweeney BJ, Edgecombe J, Churchill DR, et al. Choreoathetosis/ballismus associated with pentamidine-induced hypoglycemia in a patient with the acquired immunodeficiency syndrome. Arch Neurol. Jul 1994;51(7):723-5. [Medline].

  64. Davous P, Rondot P, Marion MH, Gueguen B. Severe chorea after acute carbon monoxide poisoning. J Neurol Neurosurg Psychiatry. Feb 1986;49(2):206-8. [Medline].

  65. Schwartz A, Hennerici M, Wegener OH. Delayed choreoathetosis following acute carbon monoxide poisoning. Neurology. Jan 1985;35(1):98-9. [Medline].

  66. Abbruzzese G, Brusa G, DallAgata D, Morena M, Spadavecchia L, Favale E. Electrophysiological analysis of motor control in patients with vascular hemichorea. Ital J Neurol Sci. Aug 1987;8(4):357-62. [Medline].

  67. Jones HR Jr, Baker RA, Kott HS. Hypertensive putaminal hemorrhage presenting with hemichorea. Stroke. Jan-Feb 1985;16(1):130-1. [Medline].

  68. Margolin DI, Marsden CD. Episodic dyskinesias and transient cerebral ischemia. Neurology. Dec 1982;32(12):1379-80. [Medline].

  69. Tabaton M, Mancardi G, Loeb C. Generalized chorea due to bilateral small, deep cerebral infarcts. Neurology. Apr 1985;35(4):588-9. [Medline].

  70. Bae SH, Vates TS Jr, Kenton EJ 3d. Generalized chorea associated with chronic subdural hematomas. Ann Neurol. Oct 1980;8(4):449-50. [Medline].

  71. Pavlakis SG, Schneider S, Black K, Gould RJ. Steroid-responsive chorea in moyamoya disease. Mov Disord. 1991;6(4):347-9. [Medline].

  72. Bruyn GW, Ferrari MD. Chorea and migraine: Hemicrania choreatica?. Cephalalgia. Jun 1984;4(2):119-24. [Medline].

  73. Kok J, Bosseray A, Brion JP, et al. Chorea in a child with Churg-Strauss syndrome. Stroke. Aug 1993;24(8):1263-4. [Medline].

  74. Kimura N, Sugihara R, Kimura A, Kumamoto T, Tsuda T. [A case of neuro-Behcets disease presenting with chorea]. Rinsho Shinkeigaku. Jan 2001;41(1):45-9. [Medline].

  75. Caviness VS Jr. Huntingtons disease. Dev Med Child Neurol. Dec 1985;27(6):826-9. [Medline].

  76. Cervera R, Asherson RA, Font J, et al. Chorea in the antiphospholipid syndrome. Clinical, radiologic, and immunologic characteristics of 50 patients from our clinics and the recent literature. Medicine (Baltimore). May 1997;76(3):203-12. [Medline].

  77. Walker FO, Hunt VP. Ballism: an association with ventriculoperitoneal shunting. Neurology. Jun 1990;40(6):1004. [Medline].

  78. Rosenblatt A, Liang KY, Zhou H, Abbott MH, Gourley LM, Margolis RL. The association of CAG repeat length with clinical progression in Huntington disease. Neurology. Apr 11 2006;66(7):1016-20. [Medline].

  79. Burton PD. Magnetic resonance imaging and brain iron: implications in the diagnosis and pathochemistry of movement disorders and dementia. Barrow Neurological Institute Quarterly. 1987;3, No. 4:15-29.

  80. Rutledge JN, Hilal SK, Silver AJ, et al. Study of movement disorders and brain iron by MR. Am J Neuroradiol. 1987;8:397-411.

  81. Montoya A, Price BH, Menear M, Lepage M. Brain imaging and cognitive dysfunctions in Huntingtons disease. J Psychiatry Neurosci. Jan 2006;31(1):21-9. [Medline].

  82. Hosokawa S, Ichiya Y, Kuwabara Y, et al. Positron emission tomography in cases of chorea with different underlying diseases. J Neurol Neurosurg Psychiatry. Oct 1987;50(10):1284-7. [Medline].

  83. Otsuka M, Ichiya Y, Kuwabara Y, et al. Cerebral glucose metabolism and striatal 18F-dopa uptake by PET in cases of chorea with or without dementia. J Neurol Sci. Apr 1993;115(2):153-7. [Medline].

  84. Tanaka M, Hirai S, Kondo S, et al. Cerebral hypoperfusion and hypometabolism with altered striatal signal intensity in chorea-acanthocytosis: a combined PET and MRI study. Mov Disord. Jan 1998;13(1):100-7. [Medline].

  85. Grove VE Jr, Quintanilla J, DeVaney GT. Improvement of Huntingtons disease with olanzapine and valproate. N Engl J Med. Sep 28 2000;343(13):973-4. [Medline].

  86. Shannon KM. Hemiballismus. Clin Neuropharmacol. Oct 1990;13(5):413-25. [Medline].

  87. Thompson TP, Kondziolka D, Albright AL. Thalamic stimulation for choreiform movement disorders in children. Report of two cases. J Neurosurg. Apr 2000;92(4):718-21. [Medline].

  88. Krauss JK, Loher TJ, Weigel R, et al. Chronic stimulation of the globus pallidus internus for treatment of non-dYT1 generalized dystonia and choreoathetosis: 2-year follow up. J Neurosurg. Apr 2003;98(4):785-92. [Medline].

  89. Moro E, Lang AE, Strafella AP, et al. Bilateral globus pallidus stimulation for Huntingtons disease. Ann Neurol. Aug 2004;56(2):290-4. [Medline].

  90. Bachoud-Levi AC, Gaura V, Brugieres P, Lefaucheur JP, Boisse MF, Maison P, et al. Effect of fetal neural transplants in patients with Huntingtons disease 6 years after surgery: a long-term follow-up study. Lancet Neurol. Apr 2006;5(4):303-9. [Medline].

  91. Keene CD, Sonnen JA, Swanson PD, Kopyov O, Leverenz JB, Bird TD, et al. Neural transplantation in Huntington disease: long-term grafts in two patients. Neurology. Jun 12 2007;68(24):2093-8. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

adult chorea, ballism, hemiballism, biballism, paraballism, ballismus, hemiballismus, biballismus, paraballismus, choreoathetosis, athetosis, benign hereditary chorea, Sydenham chorea, Sydenham's chorea, Huntington's disease, Huntington disease, HD, senile chorea, neuroacanthocytosis, Wilson disease, Wilson's disease, WD

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Stephanie M Vertrees, MD, Staff Physician, Section of Internal Medicine, Department of Neurology, Dartmouth-Hitchcock Medical Center
Stephanie M Vertrees, MD is a member of the following medical societies: American Academy of Neurology and American Medical Women's Association
Disclosure: Nothing to disclose.

Coauthor(s)

Stephen A Berman, MD, PhD, Professor, Department of Internal Medicine, Section of Neurology, Dartmouth Medical School; Chief, Neurology Service, White River Junction Veterans Medical Center
Stephen A Berman, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Medical Editor

Stephen T Gancher, MD, Adjunct Associate Professor, Department of Neurology, Oregon Health Sciences University
Stephen T Gancher, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, and Movement Disorders Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Richard J Caselli, MD, Professor, Department of Neurology, Mayo Medical School, Rochester, MN; Chair, Department of Neurology, Mayo Clinic of Scottsdale
Richard J Caselli, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Medical Association, American Neurological Association, and Sigma Xi
Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.