[Superior sagittal sinus thrombosis in paroxysmal nocturnal hemoglobinuria--an autopsied case]

A 43-year-old Japanese man was admitted to our hospital because of acute occipital headache and drowsiness. He had been diagnosed as paroxysmal nocturnal hemoglobinuria (PNH) with acute renal failure for 2 months prior to the admission. He had abdominal pain and fever of unknown origin for 2 weeks. On admission neurological examination revealed mild disturbance of consciousness, right papilledema and minimal nuchal rigidity. The urinalysis was normal and blood hemoglobin was 8.3 g/dl. Mild hemolysis was suspected because of decreased level of the serum haptoglobin. The magnetic resonance imaging (MRI) of the brain demonstrated the complete occlusion of the superior sagittal sinus by fresh thrombus and the cerebral hemorrhage in the right parieto-occipital subcortex. No other cause for cerebral sinus thrombosis was detected. The patient was treated medically using anti-edema agents, but on the twentieth hospital day he died because of brain herniation. The postmortem examination disclosed thrombosis in the superior sagittal sinus and transverse sinus on both side. There was a massive hematoma in the right parieto-occipital subcortex. The spleen and kidney also contained multiple venous thromboses. Twelve cases of cerebral sinus thrombosis with PNH have been reported in the literatures. Although cerebral sinus thrombosis is a common complication with PNH in Caucasian, but is rare in Asian including Japanese. This may probably depend upon a racial difference. To our knowledge, this is the first report of a patient with cerebral sinus thrombosis with PNH in Japan.