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Progressive oculo-orofacial-speech apraxia (POOSA).
Heidi L Roth1, Thomas A Eskin, Diane L Kendall
1Department of Neurology, University of North Carolina, Chapel Hill, North Carolina, USA.
Neurocase
|September 27, 2006
Summary
This study describes a unique neurological disorder, progressive oculo-orofacial-speech apraxia (POOSA), characterized by speech, eye, and facial movement deficits. Pathological findings reveal widespread neuronal loss, suggesting a distinct neurodegenerative condition.
Area of Science:
- Neurology
- Neuroscience
- Pathology
Background:
- Speech loss can stem from motor, language, or speech programming deficits.
- Apraxia of speech is often linked to degenerative neurological diseases.
Observation:
- A patient presented with apraxia of speech, oculo-orofacial apraxia, and supranuclear ophthalmoplegia.
- The patient showed no extrapyramidal (Parkinsonian) signs.
Findings:
- Post-mortem examination revealed neuronal loss in frontal and temporal regions.
- Significant neuronal loss and astrogliosis were observed in the basal ganglia (caudate, claustrum, globus pallidus, substantia nigra).
- Axonal loss was noted in the anterior cerebral peduncles.
Implications:
- The unique clinical and pathological profile suggests a distinct disorder, termed progressive oculo-orofacial-speech apraxia (POOSA).
- This case expands the understanding of neurodegenerative diseases affecting speech and motor control.
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