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Speech Characteristics of Patients With Pallido-Ponto-Nigral Degeneration and Their Application to Presymptomatic Detection in At-Risk Relatives

Kari Krein-Jones
Arizona State University, Tempe
Mayo Clinic, Scottsdale, AZ

Zbigniew K. Wszolek
Mayo Clinic, Jacksonville, FL

John N. Caviness
Mayo Clinic, Scottsdale, AZ

Contact author: Julie M. Liss, Motor Speech Disorders Laboratory, Department of Speech & Hearing Science, Arizona State University, Tempe, AZ 85287-0102. E-mail: julie.liss{at}asu.edu

PURPOSE: 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.

METHOD: 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.

CONCLUSION: The dysarthria of PPND is an early harbinger of disease onset. It has a mixed presentation, with hypokinetic, spastic, and flaccid features.

Key Words: dysarthria, pallido-ponto-nigral, degeneration, dementia

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