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Rapidly progressive dementia: a clinicopathologic correlation
J C Adair1, R L Schwartz, T A Eskin
1Department of Neurology, Shands Hospital, University of Florida, Gainesville, USA.
Abstract:
We evaluated a 66-year-old man with a rapidly progressive, akinetic-rigid dementia syndrome. Despite extensive testing, which included magnetic resonance imaging (MRI), we were unable to make the correct antemortem diagnosis. Autopsy demonstrated spontaneous progressive multifocal leukoencephalopathy. This report illustrates that even in the absence of characteristic MRI findings, this uncommon cause of dementia should be considered in the differential diagnosis of rapidly progressive, akinetic-rigid syndromes with dementia.
Insights
Progressive multifocal leukoencephalopathy (PML) can cause rapidly progressive dementia. This case highlights PML as a crucial consideration in differential diagnoses, even without typical magnetic resonance imaging (MRI) findings.
Area of Science:
- Neurology
- Neuroscience
- Pathology
Background:
- Rapidly progressive dementia (RPD) presents a diagnostic challenge.
- Akinetic-rigid syndromes with dementia are often associated with neurodegenerative diseases.
- Spontaneous progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease affecting the central nervous system.