A type of adult polyglucosan body disease

Polyglucosan bodies in the nervous system and other viscera are the main findings at autopsy of a 64-year-old woman who had a chronic neurologic disorder of 20 years' duration. The clinical features included muscle weakness, sensory disturbances, neurogenic bladder, dementia, and cataracts. Light and electron microscopy of the nervous system showed numerous polyglucosan bodies in the processes of neurons and astrocytes but not in neuronal perikarya. A similar clinico-pathologic presentation has been described in six previous cases. It is suggested that this is a type of adult polyglucosan body disease which probably forms a variant of Lafora's disease.