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 died suddenly within a few minutes. He had a history of severe oligophrenia, bilateral ectopia lentis requiring extraction of both lenses and operatively corrected genu valgum on both sides. In 1993 a hypergammaglobulinemia was noticed and a decortication on the left side after purulent pneumonia became necessary. Four months before death multiple abscesses in the spleen and pancreas lead to splenectomy and resection of the pancreatic tail. Furthermore, an isolated inflammatory pseudotumor (IPT) of the brain was found. Autopsy revealed as cause of death a pulmonary embolism. The pathogenesis of the cause of death and concomitant diseases will be discussed.