eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases

Chorea in Adults: Follow-up

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

Follow-up

Complications

  • The severity of the abnormal involuntary movements may cause rhabdomyolysis or local trauma in some patients.
  • The swallowing difficulties and tongue dystonia usually present in neuroacanthocytosis patients may cause aspiration pneumonia and early death in some patients.

Prognosis

Prognosis depends on the cause of the chorea. Huntington disease (HD) has a poor prognosis, because all patients will die of complications of the disease. Similarly, patients with neuroacanthocytosis may develop aspiration pneumonia, which can cause early death.

Patient Education

Genetic counseling

  • Both Huntington disease and benign hereditary chorea are inherited in an autosomal dominant pattern. Therefore, if there is a couple planning to conceive and one of the pair has Huntington disease, they should be educated that there is a 1-in-2 chance that each child they have could be affected. 
  • Wilson disease and neuroacanthocytosis both have an autosomal recessive inheritance pattern. Therefore, if both parents are carriers, there is a 1-in-4 chance that each child could be affected, and a 1-in-2 chance that each child could be a carrier. 
  • Genetic testing is available for each of these conditions. Testing should be offered to potentially affected patients and their family members, and they should be informed about the potential risk of inheriting the disease, clinical manifestations of the disease, treatment options, and prognosis. However, patients refuse to have testing for a number of reasons, and this should be respected. 

Miscellaneous

Medicolegal Pitfalls

  • In Huntington disease (HD), the quality of the genetic counseling is very important. The physician can make errors of omission and commission. The pitfalls and problems associated with genetic testing should be discussed.
    • Some individuals do not want to know ahead of time whether they will develop manifestations of the disease, but others do want to know. Still, others are unsure. Patients have committed suicide in anticipation of the onset of HD. This could lead to a lawsuit. Thus, testing should not be performed in a casual manner and not without adequate discussion.
    • On the other hand, omitting the testing or not informing patients that their offspring have a 50% chance of contracting the disease also could lead to a lawsuit.
    • If one feels uncomfortable discussing these things with the patient, the best plan may be to refer him or her to a medical geneticist who will supervise the testing.
 
Acknowledgments

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.



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
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Further Reading

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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

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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: Nothing to disclose.

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.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
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.

 
 
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