A disease in infants resembling chronic Wernicke's encephalopathy

Abstract

Summary 1.Three cases are reported of childrensuccumbing to a disease process resembling Wernicke's encephalopathy. 2.There is no convincing evidence of a dietary inadequacy of thiamine or other vitamins, nor of a difficulty with intestinal absorption, nor of an abnormally high thiamine requirement in these cases. 3.Two of the patients were siblings of a consanguineous marriage. It is suggested that a congenital metabolic disorder, possibly involving the utilization of thiamine or in the enzymatic processes with which thiamine derivatives are concerned, may be present in these cases. Summary 1.Three cases are reported of childrensuccumbing to a disease process resembling Wernicke's encephalopathy. 2.There is no convincing evidence of a dietary inadequacy of thiamine or other vitamins, nor of a difficulty with intestinal absorption, nor of an abnormally high thiamine requirement in these cases. 3.Two of the patients were siblings of a consanguineous marriage. It is suggested that a congenital metabolic disorder, possibly involving the utilization of thiamine or in the enzymatic processes with which thiamine derivatives are concerned, may be present in these cases.