| Main clinical features
|
onset in the first or second decade, with impaired nociception and progressive mutilating ulceration of the hands and feet with osteomyelitis and acroosteolysis
amputations of the hands and feet were common; sensory function was severely impaired, and nerve conduction studies showed axonal sensory, and sometimes mild motor, neuropathy, primarily in the lower limbs; sural nerve biopsies, confirmed axonal neuropathy; autonomic dysfunction included hyperhidrosis, urinary incontinence, and slow pupillary light response |