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Biochemical studies in Nevin-Jones syndrome
  1. K. Valmikinathan,
  2. J. N. Cumings
  1. Department of Chemical Pathology, Institute of Neurology, Queen Square, London

    Abstract

    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.

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