Isolated intracranial hypertension without cerebral venous sinus thrombosis in APLA syndrome: an unique association.

Abstract

Background: Antiphospholipid antibody (APLA) syndrome is an autoimmune disorder predisposing to thrombotic complications affecting CNS either by arterial vaso occlusion or venous thrombosis. Cerebral venous sinus thrombosis (CVST) secondarily produces raised intracranial pressure (ICP). However intracranial hypertension without evidence of CVST is rare entity.

Case presentation: We present two cases of elevated ICP with absence of identifiable CVST. Case 1, a 28-year-old female presented with a 2 months history of headache followed by bilateral vision loss. Cerebrospinal fluid (CSF) opening pressure and fundoscopy along with Contrast Magnetic resonance imaging (MRI) was suggestive of Idiopathic intracranial hypertension (IIH) and patient improved with acetazolamide. 5 months later she presented with acute onset right sided hemiparesis. MRI showed acute left Middle cerebral artery (MCA) territory infarct with normal contrast Magnetic resonance venography (MRV). Anti-cardiolipin and Beta 2 glycoprotein (both IgG and IgM) titres were elevated. Case 2, a 23-year-old female presented with headache and diplopia of 2 months duration. Based on CSF, fundoscopy and contrast MRI brain was diagnosed as IIH and she too responded to diuretics. 2 years later she presented with recurrence of headache and APLA profile showed elevated beta 2 glycoprotein IgG and IgA.

Conclusion: This is an important non thrombotic complication of APLA syndrome and requires further large-scale study for insight into the pathogenesis and early recognition to avoid future complications.