## Abstract This review explores the history and use of the terms essential myoclonus and myoclonie dystonia. In addition, the review proposes that herediatry essential myoclonus and dominantly inherited myoclonic dystonia, with lightning jerks and dramatic response to alcohol, are the same disease
Myoclonus-dystonia: An update
β Scribed by Kiyoka Kinugawa; Marie Vidailhet; Fabienne Clot; Emmanuelle Apartis; David Grabli; Emmanuel Roze
- Book ID
- 102506775
- Publisher
- John Wiley and Sons
- Year
- 2009
- Tongue
- English
- Weight
- 217 KB
- Volume
- 24
- Category
- Article
- ISSN
- 0885-3185
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β¦ Synopsis
Abstract
Our knowledge of the clinical, neurophysiological, and genetic aspects of myoclonusβdystonia (MβD) has improved markedly in the recent years. Basic research has provided new insights into the complex dysfunctions involved in the pathogenesis of MβD. On the basis of a comprehensive literature search, this review summarizes current knowledge on MβD, with a focus on recent findings. We also propose modified diagnostic criteria and recommendationsfor clinical management. Β© 2008 Movement Disorder Society
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## Abstract Inherited myoclonusβdystonia (MβD) is an autosomal dominant disorder characterized by myoclonus and dystonia that often improves with alcohol. To examine the electrophysiologic characteristics of MβD, we studied 6 patients from 4 different families and 9 ageβmatched healthy subjects. Ne