SUSAC Syndrome- An under reported and misdiagnosed condition that needs greater awareness for adequate management

Abstract

Background: SUSAC Syndrome is an autoimmune mediated vasculopathy that affects the very small blood vessels in the brain, retina and cochlea. Case report: We report a case of 32 year old para 2 who was a known case of SUSAC syndrome diagnosed in her second pregnancy at 28 weeks of gestation after an episode of coma and fainting. She was transferred to tertiary care with abnormal cognitive function and MRI scan that showed evidence of active vasculopathy. Her symptoms were severe headaches with flashing of lights, tinnitus and joint pains. She had multidisciplinary care with neuroinflammatory team. An emergency ceasarian section was performed at term due to fetal compromise. She had uneventful postnatal course . Discussion: Susac Syndrome affects women more than men usually between 20-40 years. It is difficult to diagnose the condition because of other overlapping symptoms therefore the true prevalence is unknown Headache is an important but often under-appreciated symptom which frequently has a migraines character and may precede other symptoms. Conclusion: A high degree of suspicion is needed to diagnose and treat women presenting in pregnancy. Women will need careful monitoring and neuro inflammatory input for better outcome.