Neuro-Behçet's presentation as cerebral venous thrombosis - A report of two cases and review of the literature

Abstract

Cerebral venous thrombosis (CVT) is a rare stroke with multiple risk factors. One rare risk factor is Behçet's disease (BD). Out of around 3000 cases at our center in the past 10 years, two cases of BD with CVT were seen. Herein, we report on their clinical symptoms, course, and management. Case 1 was a 18-year-old girl with a history of recurrent skin lesions presenting with encephalopathy syndrome due to CVT, requiring decompression. Despite our best efforts, she developed complications and expired due to sepsis. Case 2 was a 22-year-old male with raised intracranial pressure syndrome and a history of recurrent orogenital ulcers. His evaluation showed retinal vasculitis, papilledema, and bilateral lateral rectus palsy. Both had CVT on neuroimaging and had positivity for human leukocyte antigen-B51. Case 2 responded to the anticoagulation and immunomodulation. Risk factor identification is essential in managing CVT, and planned evaluation (clinical or investigations) plays an important role in identifying rare causes that need specific treatment.

Figure 1

(a) Neuroimage of Case 1: CT of the brain showing hyperdense SSS (solid white star) and bilateral frontoparietal hemorrhagic infarct (solid double-headed white arrow) along with midline shift and mass effect. (b–e) Neuroimages of Case 2. (b) Healed scrotal ulcers (solid white star). (c) Fundoscopy picture showing grade 3 papilledema (solid white arrow) and perivascular scarring (white oval). (d and e) MRI of the brain showing SSS thrombosis (solid white arrow) and bilateral perioptic nerve sheath dilation with tortuous optic nerve (solid double-headed white arrow)