Primary cerebral venous thrombosis in young adults: the diverse manifestations of an underrecognized disease

Primary cerebral venous thrombosis has a wide clinical and pathological spectrum, which has not been recognized. Seven cases of aseptic cerebral venous thrombosis (5 fatal) are discussed, with pathological study and literature review. Five patients ranged in age from 22 to 31 years. In 3 cases no discernible cause for thrombosis was found. Novel features of these cases include: primary bilateral internal cerebral venous thrombosis; isolated thrombocytopenia; occurrence in the context of (1) ulcerative colitis plus oral contraceptive use, (2) a twin transfusion syndrome, and (3) presenting syndrome five years after mastectomy for carcinoma. Diagnosis and treatment are discussed, and it is suggested that a focal coagulopathy may be responsible for this underdiagnosed condition.