Huntington’s disease with no chorea-westphal variant

Arun Gogna, Annie Kanchan Baa, Jhasaketan Meher, Vishnu Priya A R and Kruthik Raj CG

Huntington’s disease (HD) is a rare hereditary neurodegenerative disorder which is autosomal dominant and is characterized in over 90% of cases, by chorea as the presenting motor symptom along with dementia and neuropsychiatric manifestations in adults. Juvenile Huntington disease (JHD) known as Westphal variant (WV) presents itself with significantly different signs characterized mainly by rigidity and myoclonus, therefore causing diagnostic difficulties. In this paper, we present WV in a 13 year old girl.

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