Fine structural changes in the sural nerve of patients with acanthocytosis

Summary The fine structure of sural nerve biopsies is described in two brothers with normo-lipoproteinaemic acanthocytosis and an associated neurological syndrome. There was a severe reduction of myelinated fibres. The Schwann cells had an increased population of lysosomes and contained remnants of myelin. The myelin lamellae were often split at the intraperiod lines. Centrioles were found in Schwann cells, fibroblasts and endothelial cells. The significance of these findings is discussed.