title: Rapid-onset dystonia-parkinsonism in a second family.
abstract: 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.
authors.affiliation: Department of Neurology, Indiana University School of Medicine, Indianapolis, USA.
authors.forename: A
authors.initials: A
authors.lastname: Brashear
authors.forename: D
authors.initials: D
authors.lastname: DeLeon
authors.forename: S B
authors.initials: SB
authors.lastname: Bressman
authors.forename: D
authors.initials: D
authors.lastname: Thyagarajan
authors.forename: M R
authors.initials: MR
authors.lastname: Farlow
authors.forename: W B
authors.initials: WB
authors.lastname: Dobyns
mesh_terms: 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