Rapid-onset dystonia-parkinsonism in a second family.
Rapid-onset dystonia-parkinsonism (RDP), first described in a large Midwestern family, is now reported in a second, apparently unrelated, family in which four individuals have this same syndrome. All four developed sudden onset of dysarthria, dysphagia, severe dystonic spasms, bradykinesia, and postural instability over less than 1 hour to a few days. Three of the four had stable limb dystonia for several years preceding the onset of combined dystonia-parkinsonism. Treatment with levodopa/carbidopa provided little benefit. We propose diagnostic criteria for RDP and further define the spectrum of this unusual disease.
Brashear
A
A
Department of Neurology, Indiana University School of Medicine, Indianapolis, USA.
DeLeon
D
D
Bressman
S B
SB
Thyagarajan
D
D
Farlow
M R
MR
Dobyns
W B
WB
D000293:Adolescent; D000328:Adult; D002648:Child; D004421:Dystonia / Q000235:genetics* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D010300:Parkinson Disease / Q000235:genetics* / Q000503:physiopathology; D010375:Pedigree; D013577:Syndrome; D013997:Time Factors