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Pathologic heterogeneity in clinically diagnosed corticobasal degeneration
B F Boeve1, D M Maraganore, J E Parisi
1Department of Neurology, Mayo Clinic, Rochester, MN, USA.
Neurology
|September 17, 1999
Summary
Clinically diagnosed corticobasal degeneration (CBD) often presents with heterogeneous pathologies, not always including basal ganglia degeneration. Asymmetric parietofrontal cortical degeneration is the key finding, requiring tissue examination for accurate diagnosis.
Area of Science:
- Neuropathology
- Neurodegenerative diseases
- Clinical neurology
Background:
- Corticobasal degeneration (CBD) was initially considered a distinct clinicopathologic entity.
- Consistent clinical and laboratory findings led to proposals for confident in-life diagnosis.
Purpose of the Study:
- To analyze the clinicopathologic findings in a large series of clinically diagnosed CBD cases.
Main Methods:
- Retrospective review of Mayo Clinic cases (1990-1997) with clinical CBD diagnosis and autopsy.
- Standardized neuropathologic examination to determine pathology distribution and severity.
- Cases presented with progressive asymmetric rigidity and apraxia, indicating cortical and basal ganglionic dysfunction.
Main Results:
- Seven of thirteen cases were pathologically confirmed as CBD; others included Alzheimer's disease, progressive supranuclear palsy, Pick's disease, and Creutzfeldt-Jakob disease.
- Two cases lacked significant basal ganglia/nigral degeneration despite extrapyramidal signs.
- All cases exhibited focal/asymmetric parietofrontal cortical atrophy with neuronal loss and gliosis.
Conclusions:
- Clinical features of CBD are linked to diverse pathologies.
- CBD syndrome can manifest without basal ganglia and nigral degeneration.
- Asymmetric parietofrontal cortical degeneration is the consistent pathologic hallmark.
- Definitive diagnosis of CBD necessitates tissue examination.