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Primary progressive multifocal leukoencephalopathy presenting as an extrapyramidal syndrome
K P Bhatia1, J H Morris, R S Frackowiak
1University Department of Clinical Neurology, Institute of Neurology, London, UK.
Journal of Neurology
|January 1, 1996
Summary
A rare case of primary progressive multifocal leukoencephalopathy (PML) presented with parkinsonian syndrome and cognitive decline. Autopsy confirmed JC virus in the brain, revealing unusual linear lesions.
Area of Science:
- Neurology
- Neurovirology
- Pathology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease typically associated with immunosuppression.
- Parkinsonian syndromes are characterized by motor deficits like tremor and rigidity.
- Cognitive decline and visual disturbances can occur in various neurological disorders.
Observation:
- A 63-year-old woman developed a progressive neurological illness mimicking parkinsonism, including tremor, rigidity, and gait issues.
- Her condition worsened over 10 years, leading to cognitive decline, visuomotor apraxia, and visual agnosia.
- Autopsy revealed characteristic changes of PML with JC virus confirmation and atypical linear lesions at the cortico-white matter junction.
Findings:
- The patient's presentation and autopsy findings suggest a rare instance of primary PML (without known immune disorder or malignancy).
- Neuropathology showed both active and inactive ("burnt out") PML lesions, some with unusual linear morphology.
- The case demonstrates that white matter disorders, specifically PML, can manifest as a parkinsonian syndrome.
Implications:
- This case expands the known clinical spectrum of PML, particularly primary PML.
- It highlights the importance of considering PML in the differential diagnosis of parkinsonian syndromes, even in immunocompetent individuals.
- The unusual lesion morphology warrants further investigation into PML pathogenesis and its varied presentations.