DEMENTIA OF FRONTAL-LOBE TYPE DUE TO ADULT POLYGLUCOSAN BODY DISEASE

We describe a patient with adult polyglucosan body disease (APBD) who presented with a dementia of frontal lobe type (FLD), with a neurogeni c bladder but no symptoms of sensory motor peripheral neuropathy. Diag nosis was made from a cerebral biopsy specimen which showed an accumul ation of intra-axonal polyglucosan bodies in the central nervous syste m. This case differs from the usual presentation, in which gait distur bance is the main symptom and diagnosis is possible by sural nerve bio psy. Little is known about the neuropsychological pattern of APBD deme ntia but FLD has not previously been described. APBD is a heterogeneou s clinical entity of unknown cause. This diagnosis must be considered in elderly patients with dementia.