A 12-year-old boy developed a slowly progressive spastic gait at the a
ge of 3. A marked loss of pain and temperature sensations led to a mut
ilating acropathy starting at age 5. Electrodiagnostic studies reveale
d a symmetric, axonal, predominantly sensory neuropathy, and magnetic
resonance imaging ruled out compression of spinal cord. Sural nerve bi
opsy disclosed a predominant involvement of unmyelinated and a global
loss of myelinated fibers, particularly larger ones. Clinical, electro
diagnostic and pathologic findings of this case most likely represent
an example of the ''Cavanagh's variant,'' an unusual but distinct enti
ty within the hereditary sensory and autonomic neuropathies. (C) 1996
John Wiley & Sons, Inc.