SUDDEN-DEATH OF AN 18-YEAR-OLD MAN WITH HOMOCYSTINURIA AND INTRACRANIAL INFLAMMATORY PSEUDOTUMOR (IPT)

An 18-year-old mentally deficient man with well-known homocystinuria d ied suddenly within a few minutes. He had a history of severe oligophr enia, bilateral ectopia lentis requiring extraction of both lenses and operatively corrected genu valgum on both sides. In 1993 a hypergamma globulinemia was noticed and a decortication on the left side after pu rulent pneumonia became necessary. Four months before death multiple a bscesses in the spleen and pancreas lead to splenectomy and resection of the pancreatic tail. Furthermore, an isolated inflammatory pseudotu mor (IPT) of the brain was found. Autopsy revealed as cause of death a pulmonary embolism. The pathogenesis of the cause of death and concom itant diseases will be discussed. (C) 1998 Elsevier Science Ireland Lt d. All rights reserved.