Speech characteristics of patients with pallido-ponto-nigral degeneration and their application to presymptomatic detection in at-risk relatives.
OBJECTIVE
This report describes the speech characteristics of individuals with a neurodegenerative syndrome called pallido-ponto-nigral degeneration (PPND) and examines the speech samples of at-risk, but asymptomatic, relatives for possible preclinical detection.


METHODS
Speech samples of 9 members of a PPND kindred were subjected to perceptual characterization. Speech deterioration patterns were reported for 2 participants followed longitudinally at 6-month intervals. Cross-sectional findings were reported for 3 participants at various stages of disease. Longitudinal and cross-sectional findings were used to guide the examination of 4 at-risk, but asymptomatic, participants.


RESULTS
Results revealed a progressive mixed dysarthria with hypokinetic, spastic, and flaccid features. It was characterized primarily by vocal tremor and high-frequency vocal flutter, speaking rate abnormalities most often in the direction of slowing, and a tendency toward monopitch. Dysarthria progression was marked by exacerbation and increasing severity of early features, progressive decrease in spontaneous speech output, verbal perseverations, and eventual mutism. Results for at-risk participants revealed preclinical speech abnormalities that preceded other motor signs. Speech results were examined in light of available autopsy findings for site of lesion associations.


CONCLUSIONS
The dysarthria of PPND is an early harbinger of disease onset. It has a mixed presentation, with hypokinetic, spastic, and flaccid features.
Liss
Julie M
JM
Motor Speech Disorders Laboratory, Department of Speech & Hearing Science, Arizona State University, Tempe, AZ 85287-0102, USA. julie.liss@asu.edu
Krein-Jones
Kari
K
Wszolek
Zbigniew K
ZK
Caviness
John N
JN
D003430:Cross-Sectional Studies; D004401:Dysarthria / Q000209:etiology / Q000503:physiopathology*; D006801:Humans; D008137:Longitudinal Studies; D019636:Neurodegenerative Diseases / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology*; D012307:Risk Factors; D013060:Speech; D013068:Speech Production Measurement; D013637:Tape Recording