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Westphal variant Huntington disease and refractory catatonia: a case report.

Abstract
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.
AuthorsJorge Merida-Puga, Jesus Ramirez-Bermudez, Luis Carlos Aguilar-Venegas, Gregory L Fricchione, Mariana Espinola-Nadurille
JournalCognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive Neurology (Cogn Behav Neurol) Vol. 24 Issue 4 Pg. 204-8 (Dec 2011) ISSN: 1543-3641 [Electronic] United States
PMID22123587 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Dopamine Agents
  • Drug Combinations
  • Levodopa
  • Amantadine
  • Carbidopa
Topics
  • Adult
  • Amantadine (therapeutic use)
  • Carbidopa (administration & dosage, therapeutic use)
  • Catatonia (complications, drug therapy, therapy)
  • Combined Modality Therapy (methods, psychology)
  • Dopamine Agents (administration & dosage, therapeutic use)
  • Drug Combinations
  • Drug Resistance
  • Electroconvulsive Therapy (methods, psychology)
  • Female
  • Humans
  • Huntington Disease (complications, drug therapy, psychology, therapy)
  • Levodopa (administration & dosage, therapeutic use)

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