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Westphal Variant Huntington Disease and Refractory Catatonia: A Case Report

Merida-Puga, Jorge MD*; Ramirez-Bermudez, Jesus MD, MSc*,†; Aguilar-Venegas, Luis Carlos MD*; Fricchione, Gregory L. MD; Espinola-Nadurille, Mariana MD, MPH*,§

Cognitive and Behavioral Neurology: December 2011 - Volume 24 - Issue 4 - p 204–208
doi: 10.1097/WNN.0b013e318240080d
Case Reports

A young woman with Westphal variant (juvenile) Huntington disease (HD) also developed catatonia. Catatonia is an underdiagnosed psychomotor syndrome often associated with neurological and psychiatric disorders, but it has rarely been documented in patients with HD. Catatonia usually responds to standard treatment with benzodiazepines and electroconvulsive therapy; however, this patient’s catatonic syndrome did not improve until we augmented the standard treatment with amantadine and levodopa. The underlying pathophysiology and a neurochemical hypothesis of HD and catatonia can explain their comorbidity and the refractoriness of catatonia to treatment. Both conditions are linked to dysregulation of neurotransmitters in the striatocortical and corticocortical pathways. This understanding may serve as a guide for the use of nonstandard treatments. Our evidence also suggests that electroconvulsive therapy can be useful and safe in the treatment of HD.

*Departments of Neuropsychiatry

§Social Psychiatry, National Institute of Neurology and Neurosurgery, Mexico

Medical School of the National University of Mexico, Mexico

Department of Psychiatry, Massachusetts General Hospital, Boston, MA

The authors declare no conflicts of interest.

Reprints: Mariana Espinola-Nadurille, MSc, MD, National Institute of Neurology and Neurosurgery of Mexico, Insurgentes Sur 3877, Colonia La Fama, Chiapas 14269, Mexico (e-mail:

Received February 13, 2011

Accepted October 21, 2011

© 2011 Lippincott Williams & Wilkins, Inc.