An Atypical Cause For "Pseudotumor Cerebri": Superior Sagittal Sinus Thrombosis Secondary to Congenital Dysfibrinogenemia

Headache, papilledema and visual disturbances accompanied by increased intracranial pressure in the absence of an identifiable mass lesion define the diagnosis of pseudotumor cerebri. Superior sagittal sinus {SSS) thrombosis is a well-known cause of pseudotumor cerebri {PTC). SSS thrombosis is most commonly caused by contiguous infection from the meninges, sinuses or oral cavity, and less commonly from blood dyscrasias. We present the first documented case of a congenital dysfibrinogenemia as a cause of SSS thrombosis in a thin 46 year old male. The hematologic methodology arriving at this diagnosis is reviewed, as well as MRI diagnosis, including the emerging technique of phase-contrast MRI angiography. The characteristics of congenital dysfibrinogenemias and the importance of carefully searching for primary causes of SSS thrombosis are highlighted.