{"title":"[Chronic X-linked recessive bulbospinal amyotrophy (Kennedy-Stefanis type). Apropos of a case].","authors":"G Serratrice, P Guastalla","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>A new observation of chronic X-linked recessive spinal amyotrophy is reported. This series of cases fits the description of Kennedy-Stefanis chronic spinal amyotrophy, whose main characteristics are: sex-linked recessive inheritance, weakness and bulbo-spinal amyotrophy with proximal predominance, facio-lingual fasciculations, areflexia, gynecomastia in half of cases, very slowly progressive course, neurogenic EMG with normal nerve conduction velocities, and neurogenic muscle biopsy.</p>","PeriodicalId":18005,"journal":{"name":"La semaine des hopitaux : organe fonde par l'Association d'enseignement medical des hopitaux de Paris","volume":"60 14","pages":"1003-5"},"PeriodicalIF":0.0000,"publicationDate":"1984-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"La semaine des hopitaux : organe fonde par l'Association d'enseignement medical des hopitaux de Paris","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
A new observation of chronic X-linked recessive spinal amyotrophy is reported. This series of cases fits the description of Kennedy-Stefanis chronic spinal amyotrophy, whose main characteristics are: sex-linked recessive inheritance, weakness and bulbo-spinal amyotrophy with proximal predominance, facio-lingual fasciculations, areflexia, gynecomastia in half of cases, very slowly progressive course, neurogenic EMG with normal nerve conduction velocities, and neurogenic muscle biopsy.
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