Neuropsychology and PET Findings of Corticobasal Degeneration

ABSTRACT

BACKGROUND: Corticobasal degeneration (CBD) is a rare neurodegenerative disorder involving the cortical and subcortical areas enabling it to cause abnormalities of both cognition and movement. In some reports, CBD has been known to develop variable degrees of intellectual, memory and language impairment. However, detailed neuropsychological assessments of CBD up to now have been few. We conducted a detailed neuropsychological investigation and FDG-PET to delineate the pattern of neuropsychological dysfunction of CBD and to correlate neuropsychological dysfunction with FDG-PET findings. METHODS: All 6 patients (5; right handedness, 1; left handedness) were diagnosed with CBD using clinical criteria. All the patients underwent detailed neuropsychological and brain FDG-PET tests. RESULTS: Neuropsychological tests showed multiple cognitive dysfunctions with moderate memory impairment. FDG-PET scans showed asymmetric decreased metabolism in bilateral fronoto-temporo-parietal cortical and subcortical areas. The extent of asymmetricity was greater on the subcortical than cortical areas. Unlike previous reports, the medial temporal and fronto-parietal metabolisms were similarly depressed. CONCLUSIONS: All CBD patients displayed prominent deficits on mostly cognitive domains. FDG-PET may explain the relation between cognitive dysfunctions and cortical hypometabolism and help differentiate CBD with other dementing disorders.