Abstract Number ‐ 217: Case Study: Snowball strokes

Abstract

Introduction 55 year old RH female, PMH of hypothyroidism and gastric bypass who presented with complaints of 1–2 weeks of “feeling foggy in the head”, dizziness, and unsteady gait. The patient’s husband said she had delayed responses. She denied headache, vision change, or hearing loss. Exam was without focal neurologic deficit, NIHSS of 0.Though CTA head and neck were unremarkable, MRI Brain showed multiple scattered small acute or subacute infarcts involving the corpus callosum. This distribution was thought to suggest a central embolic source. The patient was admitted for stroke workup which was unrevealing including: Normal echocardiogram including bubble study. Her symptoms progressed and she was unable to find outpatient follow up. Two weeks later, she presented again but this time with progressive generalized weakness. At the time of her initial discharge, the patient was ambulatory with her walker, however upon re‐admission the patient required a wheelchair. Exam showed flaccid bilateral lower extremities, brisk reflexes, bilateral Hoffman’s and Babinski signs.Repeat MRI Brain was concerning for Susac syndrome with new lesions in the corpus callosum. CSF showed pleocytosis with markedly elevated protein (WBC 8, protein 153, glucose 55). Treatment with Solu‐Medrol 1000 mg q12h IV, then IVIG 0.4 kg/mg/day both for 5 days, Rituximab 1000mg for 2 days, then transition to prednisone 80 mg /day and CellCept 1000mg BID. She returned 14 days after with confusion, regression of prior gait improvement, and repeat MRI Brain showed additional new multifocal strokes. She repeated 3 days of 1000mg IV Solu‐Medrol, then was treated with cyclophosphamide due to clinical progression. Fluorescein angiography showed evidence of branch retinal artery occlusions bilaterally. She was placed on a prednisone taper from 80mg/day to 40mg/day over the course of the 2 months, with the plan to continue Cellcept 1000mg BID for 2 years. Methods NA‐ Single case study Results NA‐Single case study Conclusions Susac syndrome is an autoimmune disorder, affecting microvasculature of the brain, retina and cochlea. A triad of symptoms: encephalopathy, branch retinal artery occlusions, and hearing loss is classic, though only 13% have the complete triad at onset. It is often associated with headaches, psychiatric features (paranoia), bilateral cochlear hearing loss, and vertigo. It typically occurs in women 20–40 years of age. Brain MRI WO shows white‐matter disturbance, frequently mistaken for Multiple Sclerosis, however the main difference is that the corpus callosum is always affected with lesions that resemble “snowballs”, or “holes”. Treatment for Susac syndrome is immunosuppression, with high‐dose corticosteroids, also with IVIG, Mycophenolate mofetil, and Cyclophosphamide.