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Case Reports
. 2000 Nov 14;55(9):1368-75.
doi: 10.1212/wnl.55.9.1368.

The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia

A Kertesz  1 , P Martinez-Lage, W Davidson, D G Munoz
Affiliations
Case Reports

The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia

A Kertesz et al. Neurology. .

Abstract

Objective: To provide evidence for the hypothesis that the corticobasal degeneration syndrome (CBDs) overlaps significantly with primary progressive aphasia and frontotemporal dementia, and that CBDs is part of the Pick complex.

Background: Corticobasal degeneration has been mainly described as a movement disorder, but cognitive impairment is also increasingly noted.

Methods: Thirty-five cases of clinically diagnosed CBDs were followed-up with clinical, neuropsychological, and neuroimaging investigations. Twenty-nine patients were seen prospectively in movement disorder and cognitive neurology clinics; five of these came to autopsy. Six other autopsied cases that fulfilled the clinical criteria of CBDs were added with retrospective review of records.

Results: All 15 patients presenting with movement disorders developed behavioral, cognitive, or language deficits shortly after onset or after several years. Patients presenting with cognitive problems (n = 20), progressive aphasia (n = 13), or frontotemporal dementia (n = 7) developed the movement disorder subsequently. Eleven cases with autopsy had CBD or other forms of the Pick complex.

Conclusions: There is a clinical overlap between CBD, frontotemporal dementia, and primary progressive aphasia. There is also a pathologic overlap between these clinical syndromes. The recognition of this overlap will facilitate the diagnosis and avoid consideration of CBD as "heterogenous."

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