DEMENTIA OF ADULT POLYGLUCOSAN BODY DISEASE - EVIDENCE OF CORTICAL AND SUBCORTICAL DYSFUNCTION

Objectives: To characterize the dementia associated with adult polyglu cosan body disease (APBD) and to correlate the cognitive deficits with abnormalities found on magnetic resonance imaging (MRI). Methods: Qua ntitative neuropsychological testing and MRI in one man with APBD and a review of the literature. Results: The dementia of APBD affects cort ical and subcortical functions. The cognitive deficits correlate with MRI findings of cortical atrophy and white-matter abnormalities. Concl usion: Neuropsychological testing and MRI are helpful in the evaluatio n of patients with APBD.