POSTOPERATIVE PSYCHOSIS IN HOMOCYSTINURIA

Young homocystinuria patients suffering from lens dislocation frequent ly have to undergo eye surgery. We describe a 16-year-old girl with mi ld mental retardation who became psychotic-delirant immediately after the last of three lentectomia operations performed under general thiop ental anaesthesia. Because methionine, hemocysteine, its oxidation pro duct homocysteate and cysteine are potent glutamate agonists, the dist urbance of the sulphur containing amino acid (SCAA) metabolism in homo cystinuria patients may alter the function of cerebral glutamatergic t ransmission. The chronic and acute neurological and psychiatric sympto ms of homocystinuria patients offer a clue to studies of the neurotoxi c but also antipsychotic potency of glutamate agonists like the SCAAs in humans.