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NEUROLOGY 1993;43:742
© 1993 American Academy of Neurology

Cerebral venous thrombosis in paroxysmal nocturnal hemoglobinuria

Report of two cases Mazen Al-Hakim, MD, M. Bashar Katirji, MD, Ivan Osorio, MD and Russell Weisman, MD

Department of Neurology (Drs. Al-Hakim, Katirji, and Osorio) and Division of Hematology, Department of Medicine (Dr. Weisman), Case Western Reserve University and University Hospitals of Cleveland, Cleveland, OH.

We report the clinical, radiologic, and postmortem findings in two patients with paroxysmal nocturnal hemoglobinuria (PNH) who developed cerebral venous thromboses (CVTs). In contrast with those in most published cases, our patients did not have focal neurologic signs. Antemortem diagnosis of CVT had been made by MR cerebral venograms. We conclude that (1) PNH should be considered in any patient with stroke associated with iron deficiency anemia, hemolysis, hemoglobinuria, or hemosiderinuria; (2) PNH should be in the differential diagnosis of CVT; (3) the latter could present without focal neurologic signs; and (4) MR cerebral venography may be a reliable diagnostic alternative to cerebral angiography when CVT is suspected.

Presented at the 44th annual meeting of the American Academy of Neurology, San Diego, CA, May 1992.

Address correspondence and reprint requests to Dr. Mazen Al-Hakim, Department of Neurology, University Hospitals of Cleveland, 2074 Abington Road, Cleveland, OH 44106.

Received July 7, 1992. Accepted for publication in final form August 19, 1992.