Intracranial plasma cell granuloma and homocystinuria

A rare case of plasma cell granuloma (PCG) of the brain is reported. An 18-year-old man with a well known homocystinuria and a history of severe oligophrenia, grand-mal-epilepsia, bilateral ectopia lentis requiring extraction of both lens and operatively correlated genu valgum, died suddenly within a few minutes. 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 led to splenectomy and resection of the pancreatic tail. Autopsy revealed a pulmonary embolism and an isolated intracranial plasma cell granuloma of the left hemisphere.