| Main clinical features
|
late-onset, slowly progressive disorder affecting the central and peripheral nervous systems; onset after age 40 years with a variable combination of cognitive impairment, pyramidal tetraparesis, peripheral neuropathy, and neurogenic bladder; other manifestations include cerebellar dysfunction and extrapyramidal signs
loss of vibration sense in the lower limbs associated with an axonal sensorimotor polyneuropathy with reduced compound motor action potentials; sural nerve biopsy showed abundant intraaxonal polyglucosan bodies |