Dementia as the most common presentation of cortical-basal ganglionic degeneration

Objective: To evaluate the clinical presentations and dominant symptoms of patients with postmortem proven cortical-basal ganglionic degeneration (CBG D) from one neuropathology center. Background: CBGD is a rare but increasin gly recognized condition with clinical and pathologic features that continu e to evolve. Attempts have been made to develop clinical criteria to enhanc e the specificity of diagnosis, but it is not clear what proportion of pati ents harboring CBGD disease present in the "classical" fashion versus other presentations. Previous large-case series that emphasize a parietal/percep tual-motor presentation may be biased because the cases mainly originate fr om movement disorder centers. Methods: Thirteen cases of pathologically con firmed CBGD with sufficient clinical data were identified from a single neu ropathology center between 1981 and 1996. Results: Before death, only 4 of the 13 patients had a clinical diagnosis of CBGD, 6 had a clinical diagnosi s of Alzheimer's disease (AD), 1 had AD and parkinsonism, and 2 had an atyp ical dementia of the frontotemporal type. Nine of 13 cases had early dement ia. Conclusions: Dementia was the most common presentation of CBGD in this study. Despite the best efforts to define criteria to enhance the specifici ty of a diagnosis of CBGD, it is becoming clear that the clinical syndrome that accompanies this disease is quite varied. Unfortunately, patients fulf illing classical diagnostic criteria may represent a minority of those with this pathologic diagnosis.