Quantitative histologic study of the sural nerve in Lesch-Nyhan syndrome.
 The sural nerve of a 31-year-old man with Lesch-Nyhan syndrome obtained at autopsy was studied histologically.
 Large, myelinated nerve fibers were reduced in number and no myelinated nerve fibers larger than 5 microns were seen.
 Diameter distribution of myelinated nerve fibers did not demonstrate a bimodal pattern.
 The density of myelinated nerve fibers was 5,530/mm2 and was decreased as compared to the controls.
 On electron microscopic examination, lipid-like inclusions were observed in the cytoplasm of some Schwann cells.
 The role of these inclusions could not be elucidated, but reduction of larger myelinated nerve fibers suggests a peripheral nervous disorder in patients with this syndrome; therefore, patients with Lesch-Nyhan syndrome must be reappraised for disorders of the peripheral nervous system.
