Unusual cerebral manifestations in hereditary fructose intolerance

P. Labrune, S. Chatelon, P. Huguet and M. Odievre
Service de Pediatrie, Hopital Antoine Beclere, Clamart, France.

Five children with hereditary fructose intolerance developed symptoms of neurological impairment. In three of them, neurological involvement was related to the acute hepatic toxicity of fructose (hypoglycemia, abnormal coagulation, cardiovascular collapse); in the other two, such a relationship could not be demonstrated. Neurological impairment is not classic in hereditary fructose intolerance, but its occurrence in the acute phase of the disease is possible and does not constitute an argument against the diagnosis.