Frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17): PPND family. A longitudinal videotape demonstration.
Frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17), characterized by an autosomal dominant inheritance pattern, has recently been recognized as a distinct entity that can display a confusingly broad clinical phenotype. The pallido-ponto-nigral degeneration (PPND) variant is the prototypical example of the parkinsonism-predominant pattern of FTDP-17. A longitudinal videotape demonstration of the clinical progression of this entity in a single individual, along with brief videotape segments from three additional affected individuals, is presented in order to facilitate recognition of this disorder.
Wszolek
Z K
ZK
Department of Neurology, Mayo Clinic, Jacksonville, Florida, USA.
Kardon
R H
RH
Wolters
E C
EC
Pfeiffer
R F
RF
D000328:Adult; D002886:Chromosomes, Human, Pair 17; D003704:Dementia / Q000175:diagnosis / Q000235:genetics*; D005500:Follow-Up Studies; D008040:Genetic Linkage / Q000235:genetics*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D020734:Parkinsonian Disorders / Q000175:diagnosis / Q000235:genetics*; D014743:Videotape Recording