Adult onset myoclonic Huntington's disease

F. Carela, V. Scaioli, C. Ciano, S. Binelli, D. Oliva, F. Girotti

Research output: Contribution to journalArticle

Abstract

A patient with adult onset Huntington's disease (HD) and prominent action myoclonus is described. Neither epileptiform activity nor electroencephalography (EEG) correlates of the movements was found. Unlike the case with most (nonmyoclonic) HD patients, centro-parietal components of somatosensory evoked potentials (SEPs) were well defined and a clear V2 response was found. Treatment with valproic acid greatly reduced myoclonus suggesting that the γ-aminobutyric acid (GABA) system might be involved in the pathophysiology of myoclonus in HD.

Original languageEnglish
Pages (from-to)201-205
Number of pages5
JournalMovement Disorders
Volume8
Issue number2
Publication statusPublished - 1993

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Myoclonus
Huntington Disease
Aminobutyrates
Somatosensory Evoked Potentials
Valproic Acid
gamma-Aminobutyric Acid
Electroencephalography
Therapeutics

Keywords

  • Huntington's disease
  • Myoclonus

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Adult onset myoclonic Huntington's disease. / Carela, F.; Scaioli, V.; Ciano, C.; Binelli, S.; Oliva, D.; Girotti, F.

In: Movement Disorders, Vol. 8, No. 2, 1993, p. 201-205.

Research output: Contribution to journalArticle

Carela, F, Scaioli, V, Ciano, C, Binelli, S, Oliva, D & Girotti, F 1993, 'Adult onset myoclonic Huntington's disease', Movement Disorders, vol. 8, no. 2, pp. 201-205.
Carela, F. ; Scaioli, V. ; Ciano, C. ; Binelli, S. ; Oliva, D. ; Girotti, F. / Adult onset myoclonic Huntington's disease. In: Movement Disorders. 1993 ; Vol. 8, No. 2. pp. 201-205.
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