Speech characteristics of patients with pallido-ponto-nigral degeneration and their application to presymptomatic detection in at-risk relatives

Julie Liss, Kari Krein-Jones, Zbigniew K. Wszolek, John N. Caviness

    Research output: Contribution to journalArticle

    5 Citations (Scopus)

    Abstract

    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.

    Original languageEnglish (US)
    Pages (from-to)226-235
    Number of pages10
    JournalAmerican Journal of Speech-Language Pathology
    Volume15
    Issue number3
    DOIs
    StatePublished - Aug 2006

    Fingerprint

    Substantia Nigra
    Dysarthria
    Muscle Spasticity
    physical disability
    Mutism
    Disease
    Tremor
    speaking
    Autopsy
    examination

    Keywords

    • Dementia
    • Dysarthria
    • Pallido-ponto-nigral degeneration

    ASJC Scopus subject areas

    • Rehabilitation
    • Health Professions(all)
    • Linguistics and Language

    Cite this

    Speech characteristics of patients with pallido-ponto-nigral degeneration and their application to presymptomatic detection in at-risk relatives. / Liss, Julie; Krein-Jones, Kari; Wszolek, Zbigniew K.; Caviness, John N.

    In: American Journal of Speech-Language Pathology, Vol. 15, No. 3, 08.2006, p. 226-235.

    Research output: Contribution to journalArticle

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