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.