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Early progressive supranuclear palsy: pathology and clinical presentation
1Department of Pathology, University of Colorado Health Sciences Center, Denver 80262.
Clinical Neuropathology
|March 1, 1989
Summary
Early progressive supranuclear palsy (PSP) may present without gaze abnormalities, challenging diagnosis. Neuropathology reveals distinct changes in specific brainstem nuclei, even with subtle early symptoms.
Area of Science:
- Neuropathology
- Neurodegenerative Diseases
Background:
- Progressive supranuclear palsy (PSP) is characterized by supranuclear ophthalmoplegia, pseudobulbar palsy, rigidity, and dementia, with gaze palsy as a hallmark.
- Diagnosis is often delayed due to subtle and variable early symptoms, and reluctance to consider PSP without visual deficits.
Observation:
- Two autopsy cases presented with dementia, gait disturbances, and/or dysarthria, but lacked reported eye findings.
- Symptoms were initially misattributed to metastatic cancers.
Findings:
- Autopsy revealed prominent globose neurofibrillary tangles, cell loss, microglial nodules, and neuronophagia in the locus ceruleus, cranial nerve nuclei (III), nucleus supratrochlearis, nucleus centralis superior, and nucleus basalis of Meynert.
- Mild pallor of the globus pallidus and cerebellar dentate nucleus cell loss were noted, with sparing of the superior colliculus.
- These findings supported a diagnosis of early PSP, detailing limited neuropathologic changes.
Implications:
- Highlights that early clinical symptoms of PSP may not include gaze palsies.
- Emphasizes the need to consider PSP in the differential diagnosis for patients with gait disturbances, dementia, or dysarthria, even without ocular motor deficits.
- Documents PSP in association with carcinoma in two cases, suggesting potential co-occurrence.