CENTRAL PONTINE MYELINOLYSIS ASSOCIATED WITH ACQUIRED FOLATE-DEPLETION

After long-standing malnutrition a 15-month-old boy with signs of kwas hiorkor was admitted in a moribund state with serious hyponatraemic de hydration, hypothermia, somnolence, and signs of a pontine disconnecti on syndrome. Folic acid levels were below the detection level in the p resence of normal cobalamin levels, MRI of She brain showed global vol ume loss and signal abnormalities on the T-2-weighted images suggestiv e for central pontine myelinolysis (CPM), Brainstem acoustic evoked re sponses have remained normal. The serious metabolic and nutritional de rangements required substitution of folic acid, vitamins and trace ele ments as well as slow correction of hyponatraemic dehydration with ret urn of the sodium level over a period of four days. This therapeutic r egimen resulted in complete neurological recovery. Follow-up MRI docum ented normalisation of the initial pathologic findings, The hypothesis was put forward linking the pathogenesis of CPM with the combination of folate depletion and superimposed hyponatraemic dehydration. The pr eviously acquired folate depletion could affect normal appositional fu nction of myelin basic protein molecules due to insufficient methylati on of arginine in position 107, The subsequent development of intramye linic edema and CPM will then be triggered by the superimposed hyponat raemic dehydration, The Verification of this hypothesis requires furth er investigations.