Biochemical studies in Nevin-Jones syndrome.

The lipid profiles of the brain in four cases of Nevin-Jones syndrome are described. A loss of phosphatidyl ethanolamine was found in both white matter and cortex in two cases as well as a more general loss of lipids in the cortex in one patient; there was a normal cerebrosidesulphatide ratio in myelin. These findings indicate a general loss of myelin over a period of time. The distribution of fatty acids in cerebrosides and sulphatides show a slight reduction in nervonic acid (24:1). The findings support the view that a secondary demyelination is present.