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The studied pedigree comprises seven affected members in three generations. Their clinical picture consisted of cerebellar ataxia, pyramidal signs, facial myokymia, and ophthalmoplegia. The proband was a 39\u2010year\u2010old man in whom such a clinical picture, 5 years after onset at age 29, evolved to severe SMS and widespread myokymia. Electrophysiological study revealed continuous muscle activity in proximal limb muscles. Molecular study demonstrated the <jats:italic>MJD<\/jats:italic> gene mutation in all four examined patients with 73 to 76 CAG repeats in the expanded allele. We conclude that an excess of motor unit activity including stiff man\u2013like syndrome and widespread myokymia may be an integral part of the SCA3 clinical spectrum. \u00a9 2006 Movement Disorder Society<\/jats:p>","DOI":"10.1002\/mds.20865","type":"journal-article","created":{"date-parts":[[2006,6,15]],"date-time":"2006-06-15T07:49:37Z","timestamp":1150357777000},"page":"1031-1035","source":"Crossref","is-referenced-by-count":18,"title":["Stiff man\u2013like syndrome and generalized myokymia in spinocerebellar ataxia type 3"],"prefix":"10.1002","volume":"21","author":[{"given":"Jos\u00e9","family":"Berciano","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jon","family":"Infante","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonio","family":"Garc\u00eda","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carmen","family":"De 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