Interstitial Hypertrophic Neuropathy

Peripheral nerve tissue of a patient with an electron microscopically verified interstitial hypertrophic neuropathy contained excessive amounts of water and protein but less lipid than comparable control tissue. In the tissue hydrolyzate, hydroxyproline was increased fourfold, hexosamines, twofold. The reduction of lipid was associated with higher than normal levels of cholesteryl esters. The fatty acid profile of the triglycerides, cholesteryl esters, and phospholipids showed abnormally high concentrations of stearic acid and increased stearic acid-oleic acid ratios. The lipids of myelin isolated from the diseased peripheral nerve had most of the characteristics of human peripheral myelin (lower cholesterol and galactolipid than central nervous system myelin) but differed by a higher concentration of choline phosphoglycerides.