Adult polyglucosan body disease: clinical and nerve biopsy findings in two cases

Ann Neurol. 1983 Apr;13(4):440-4. doi: 10.1002/ana.410130411.


Two women suffered from a similar, slowly progressive illness characterized by upper and lower motor neuron involvement, sensory disturbances, and neurogenic bladder. Radiographic examinations showed cerebral atrophy and, in one, changes on computerized tomography suggesting leukodystrophy. Sural nerve biopsies revealed axonal neuropathy associated with a large number of polyglucosan bodies accumulating predominantly but not exclusively in myelinated axons.

Publication types

  • Case Reports

MeSH terms

  • Axons / pathology
  • Biopsy
  • Central Nervous System Diseases / pathology*
  • Female
  • Humans
  • Inclusion Bodies / pathology
  • Middle Aged
  • Peripheral Nervous System Diseases / pathology*
  • Sural Nerve / pathology