Chorea in Adults Clinical Presentation

  • Author: Stephanie M Vertrees; Chief Editor: Selim R Benbadis, MD   more...
 
Updated: Feb 3, 2012
 

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 disease[1, 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 disease[27, 28, 45]
    • 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.
  • Neuroacanthocytosis[1, 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 chorea[46, 47, 48]
    • 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 chorea[49, 50, 51]
    • 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 chorea[1, 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.
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Physical

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

  • Huntington disease[1, 52, 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.
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Contributor Information and Disclosures
Author

Stephanie M Vertrees  MD, Fellow in Public Health, Weill Cornell Medical College-Hospital for Special Surgery Fellowship in Medical Ethics; Fellow in Neuromuscular Medicine, Hospital for Special Surgery

Stephanie M Vertrees 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, MBA  Professor of Neurology, University of Central Florida College of Medicine

Stephen A Berman, MD, PhD, MBA is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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, Tampa General Hospital, University of South Florida College of Medicine

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: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Eric Dinnerstein, MD, Maria Alejandra Herrera, MD, and Nestor Galvez-Jimenez, MD, MSc, MHA, to the development and writing of this article.

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