The Lewis family revisited: no evidence for autosomal dominant multiple system atrophy

José Berciano; Gregor K Wenning

doi:10.1016/j.parkreldis.2005.06.001

The Lewis family revisited: no evidence for autosomal dominant multiple system atrophy

Parkinsonism Relat Disord. 2005 Sep;11(6):363-5. doi: 10.1016/j.parkreldis.2005.06.001.

Authors

José Berciano¹, Gregor K Wenning

Affiliation

¹ Department of Neurology, University Hospital Marqués de Valdecilla (UC), Santander, Spain.

PMID: 16103001
DOI: 10.1016/j.parkreldis.2005.06.001

Abstract

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.

Publication types

Case Reports
Research Support, Non-U.S. Gov't

MeSH terms

Adult
Autonomic Nervous System Diseases / genetics
Autonomic Nervous System Diseases / physiopathology
Family
Female
Humans
Hypotension, Orthostatic / genetics
Hypotension, Orthostatic / physiopathology
Machado-Joseph Disease / classification
Machado-Joseph Disease / genetics*
Male
Middle Aged
Multiple System Atrophy / classification
Multiple System Atrophy / genetics*
Shy-Drager Syndrome / classification
Shy-Drager Syndrome / genetics*