Probable multiple system atrophy in a German family.
Multiple system atrophy (MSA) is a neurodegenerative disorder of unknown aetiology. A possible underlying genetic component has not yet been identified. A family is reported with phenotypic MSA and probable autosomal dominant inheritance. The patients presented initially with either parkinsonian or cerebellar signs, and developed severe autonomic failure and typical atrophy of the brain stem and cerebellum in the course of the disease.
Wüllner
U
U
Department of Neurology, UKB, Sigmund Freud Strasse, Bonn, Germany. wuellner@uni-bonn.de
Abele
M
M
Schmitz-Huebsch
T
T
Wilhelm
K
K
Benecke
R
R
Deuschl
G
G
Klockgether
T
T
D000328:Adult; D000368:Aged; D001479:Basal Ganglia / Q000473:pathology; D001480:Basal Ganglia Diseases / Q000473:pathology; D001933:Brain Stem / Q000473:pathology; D018450:Disease Progression; D005190:Family; D005260:Female; D005858:Germany / Q000453:epidemiology; D005911:Gliosis / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000453:epidemiology / Q000235:genetics* / Q000473:pathology; D010375:Pedigree; D010641:Phenotype; D011699:Putamen / Q000473:pathology