Rare disease but increasing in diagnosis because of superior diagnostic imaging techniques.
Prior series mortality rate 30-50%.
Contemporary series reported mortality rate 8-14%.
Some patients present with catastrophic findings of stroke with neurological findings of coma, many present mild nonspecific symptoms including headache, papilledema, and intracranial hypertension.
Literature review of 144 cases revealed an acute phase mortality of 5.6% and a 9.4% mortality at the end of the follow-up period.
Acute phase deaths were most commonly due to cerebral herniation and late phase deaths were due to secondary conditions such associated malignancy rather than associated complications of the cerebral vein thrombosis itself.
Most patients that survive recover completely or have only mild functional or cognitive problems.
Fewer than 1 in 5 patients that survive have dependency or permanent disabilities at the end of a follow-up period.
Adverse outcome associated with age greater than 37 years, presence of altered consciousness at presentation, presence of focal neurological defects at presentation, intracranial hemorrhages, cortical vein involvement and presence of underlying malignancy.