Probable adult polyglucosan body disease

Adult polyglucosan body disease is a clinicopathologic entity characterized by progressive upper and lower motor neuron dysfunction, sensory loss in t he lower extremities, sphincter dysfunction, and occasionally dementia, Pat hologically, numerous large polyglucosan bodies are noted in peripheral ner ves, cerebral hemispheres, and the spinal cord, as well as in other systemi c tissues. We present a case of probable adult polyglucosan body disease ba sed on clinical history and examination, magnetic resonance images, and sur al nerve biopsy findings.