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Otto Appenzeller, MD, PhD; Mario Kornfeld, MD; Russell Snyder, MD

Arch Neurol. 1976;33(11):733-738.

Abstract
• 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.

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

Footnotes
Accepted for publication Feb 2, 1976.

Reprint requests to Department of Neurology, University of New Mexico School of Medicine, Albuquerque, NM 87131 (Dr Appenzeller).

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