The neuropathy of abetalipoproteinemia

Abstract

The clinical, electrophysiologic, and pathologic features of the peripheral neuropathy in abetalipoproteinemia (Bassen-Kornzweig disease) have been described incompletely. On the basis of findings in threee patients, we believe that large muscle fibers in peripheral nerves are selectively affected and that weakness may be due to chronic partial denervation.