In this study we evaluated the relationship between polyglucosan bodie
s and peripheral nerve lesions. The biopsied sural nerve from a patien
t with late-onset chronic sensori-motor neuropathy showed many intra-a
xonal polyglucosan bodies and segmental demyelination/remyelination. T
he formation of Schwann cell hyperplasia around the demyelinated axons
was found at the sites of polyglucosan bodies. These findings suggest
that demyelinating neuropathy is a part of the spectrum of the diseas
es characterized by the accumulation of polyglucosan bodies within cel
lular compartments.