ACROMUTILATING, PARALYZING NEUROPATHY WITH CORNEAL ULCERATION IN NAVAJO CHILDREN

Four Navajo children had a mutilating neuropathy with severe motor involvement. The disorder appeared to be recessively inherited and was present from the earliest observable age. Manifestations included severe anesthesia, corneal ulceration, painless fractures, acral mutilation and weakness. Mental function was normal. Sural nerves were practically devoid of myelinated fibers that show no evidence of regeneration. Unmyelinated axons showed degenerative and regenerative morphologic and histometric features. Onion bulb formation was absent. This neuropathy is probably a distinct clinical entity.