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NEUROLOGY 1985;35:1279
© 1985 American Academy of Neurology

Peripheral neuropathy in abetalipoproteinemia

Alison Wichman, MD, Fritz Buchthal, MD, Gholam H. Pezeshkpour, MD and Richard E. Gregg, MD

From the National Institutes of Health, NINCDS (Drs. Wichman and Buchthal), MDB, NHLBI (Dr. Gregg), Bethesda, MD, and the Armed Forces Institute of Pathology (Dr. Pezeshkpour), Washington, DC.

We studied the peripheral neuropathy of three sisters with abetalipoproteinemia. Clinically, a sensory neuropathy progressively increased in severity. There was a diminution in the amplitude of sensory action potentials and a slight-to-moderate slowing in maximum sensory conduction velocity, initially most marked in distal portions of the nerves. Motor conduction was normal, although EMG indicated subclinical signs of partial chronic denervation. The surely nerves showed a decreased number of large fibers (> 7 µm); in the patient with the neuropathy of shortest duration, small fibers and clusters of regenerating fibers indicated regeneration. In the two patients with advanced neuropathy, one-half the segments of teased fibers showed paranodal demyelination. Also, unmyelinated fibers showed evidence of regeneration.

Address correspondence and reprint requests to Dr. Wichman, NIH, Building 10, Room 5N226, Bethesda. MD 20205

This paper was presented in part at the thirty-sixth annual meeting of the American Academy of Neurology, Boston, MA, April 1984.

Accepted for publication December 6, 1984.




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