Speech Characteristics of Patients With Pallido-Ponto-Nigral Degeneration and Their Application to Presymptomatic Detection in At-Risk Relatives 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 ... Clinical Focus
Clinical Focus  |   August 01, 2006
Speech Characteristics of Patients With Pallido-Ponto-Nigral Degeneration and Their Application to Presymptomatic Detection in At-Risk Relatives
 
Author Affiliations & Notes
  • Julie M. Liss
    Arizona State University, Tempe
  • 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@asu.edu
Article Information
Speech, Voice & Prosodic Disorders / Dysarthria / Special Populations / Genetic & Congenital Disorders / Speech, Voice & Prosody / Clinical Focus
Clinical Focus   |   August 01, 2006
Speech Characteristics of Patients With Pallido-Ponto-Nigral Degeneration and Their Application to Presymptomatic Detection in At-Risk Relatives
American Journal of Speech-Language Pathology, August 2006, Vol. 15, 226-235. doi:10.1044/1058-0360(2006/021)
History: Received August 14, 2005 , Accepted February 13, 2006
 
American Journal of Speech-Language Pathology, August 2006, Vol. 15, 226-235. doi:10.1044/1058-0360(2006/021)
History: Received August 14, 2005; Accepted February 13, 2006
Web of Science® Times Cited: 3

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.

Acknowledgments
We thank the PPND patients and their families. We also gratefully acknowledge the Neurogenetic and Neuropathology laboratories at the Mayo Clinic in Jacksonville. Support for this study came from Arizona State University and the Mayo Foundation from both Scottsdale and Jacksonville. We are indebted to Audrey Strongosky for her assistance.
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