Complications

See the list below:

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.

Genetic counseling

See the list below:

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.

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Pradeep C Bollu, MD Associate Professor of Neurology, Vice Chair of Quality, Department of Neurology, Prisma Health, University of South Carolina School of Medicine, Columbia

Pradeep C Bollu, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, Boone County Medical Society, Indian Medical Association, Indian Red Cross Society, International Parkinson and Movement Disorder Society, Missouri State Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Tejas R Mehta, MBBS Resident Physician, Department of Neurology, University of Missouri-Columbia School of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

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

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Chief Editor

Selim R Benbadis, MD Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida Morsani 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, American Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Bioserenity, Ceribell, Eisai, Jazz, LivaNova, Neurelis, Neuropace, SK life science, Sunovion, Takeda, UCB<br/>Serve(d) as a speaker or a member of a speakers bureau for: Aquestive, Bioserenity, Eisai, Jazz, LivaNova, Neurelis, SK life science, Stratus, Sunovion, UCB<br/>Received research grant from: Cerevel Therapeutics, Ovid Therapeutics, Neuropace, Greenwich Jazz, SK Life Science, Xenon Pharmaceuticals, UCB, Marinus, Neuroelectrics Corporation, Longboard, Janssen, Equilibre Biopharmaceuticals.

Additional Contributors

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, Phi Beta Kappa

Disclosure: Nothing to disclose.

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, International Parkinson and Movement Disorder Society

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

Stephanie M Vertrees, MD is a member of the following medical societies: American Academy of Neurology, American Medical Women's Association

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference 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.