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November 1976

Acromutilating, Paralyzing Neuropathy With Corneal Ulceration in Navajo Children

Author Affiliations

From the departments of neurology (Drs Appenzeller and Snyder) and pathology (Dr Kornfeld), University of New Mexico School of Medicine, Albuquerque.

Arch Neurol. 1976;33(11):733-738. doi:10.1001/archneur.1976.00500110001001

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

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