The Lewis family revisited: no evidence for autosomal dominant multiple system atrophy.
In 1964, Lewis reported a familial ataxia-dysautonomia syndrome reminiscent of Shy-Drager syndrome subsequently known as multiple system atrophy (MSA). Here we review this report and propose that the Lewis family may represent an unusual form of autosomal dominant cerebellar ataxia type I, which might be categorized either as SCA3 (Machado-Joseph disease) or a new SCA subtype. There remains no conclusive evidence to support the notion of hereditary MSA.
Berciano
José
J
Department of Neurology, University Hospital Marqués de Valdecilla (UC), Santander, Spain.
Wenning
Gregor K
GK
D000328:Adult; D001342:Autonomic Nervous System Diseases / Q000235:genetics / Q000503:physiopathology; D005190:Family; D005260:Female; D006801:Humans; D007024:Hypotension, Orthostatic / Q000235:genetics / Q000503:physiopathology; D017827:Machado-Joseph Disease / Q000145:classification / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000145:classification / Q000235:genetics*; D012791:Shy-Drager Syndrome / Q000145:classification / Q000235:genetics*