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NEUROLOGY 1990;40:245
© 1990 American Academy of Neurology

Giant axonal neuropathy with inherited multisystem degeneration in a Tunisian kindred

Mongi Ben Hamida, MD, Fayçal Hentati, MD and Christiane Ben Hamida, PhD

Institut National de Neurologie, Tunis, Tunisie.

We describe a large kindred of 6 patients with a slowly progressive autosomal recessive form of giant axonal neuropathy (GAN). The propositus presented with progressive infantile onset of distal amyotrophy of 4 limbs, brisk reflexes, diffuse fasciculations, bulbar signs, and deep sensory loss in both lower limbs. The EMG and nerve biopsy showed typical hypertrophie neuritis. In 4 patients, there were giant axons filled with neurofilaments, with normal conduction velocity. In the youngest boy, the neurologic deficit was less severe, and the nerve biopsy revealed only a few unmyelinated axons filled with neurofilaments. These cases appear to represent a different genetic defect from other reported cases of GAN.

Address correspondence and reprint requests to Dr. Mongi Ben Hamida, Institut National de Neurologie, La Rabta 1007, Tunis, Tunisie.

Received April 4, 1989. Accepted for publication in final form August 1, 1989.




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