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 the lower extremities, sphincter dysfunction, and occasionally dementia. Pathologically, numerous large polyglucosan bodies are noted in peripheral nerves, cerebral hemispheres, and the spinal cord, as well as in other systemic tissues. We present a case of probable adult polyglucosan body disease based on clinical history and examination, magnetic resonance images, and sural nerve biopsy findings.
Klein
C M
CM
Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.
Bosch
E P
EP
Dyck
P J
PJ
D017668:Age of Onset; D017809:Fatal Outcome; D005936:Glucans; D006801:Humans; D002479:Inclusion Bodies; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D019636:Neurodegenerative Diseases / Q000473:pathology*; D013497:Sural Nerve / Q000473:pathology
D005936:Glucans