Chaminda Kumara*, Janaka Peiris, Mary Muthukumarasamy and Mahinda Weerasekara
National Hospital, Kandy, Sri Lanka
*Corresponding Author: Chaminda Kumara, National Hospital, Kandy, Sri Lanka.
Received: November 22, 2022; Published: December 08, 2022
Background: Susac syndrome is an exceedingly rare disease which is described as a typical triad of clinical manifestations, sensorineural hearing loss, encephalopathy, and branch retinal artery occlusion. It is infrequent to get all 3 clinical features in the start of illness. Susac syndrome was not reported before in Sri Lanka.
Case Presentation: A 32-Year-old apparently healthy female patient presented with severe headache for 3 days. She became drowsy and confused later. A history of left side hearing impairment was also reported. On examination she was drowsy and found to have generalized hyperreflexia with bilateral extensor plantar response. Magnetic resonance imaging of brain revealed classical “snowball lesions” in the corpus callosum while internal capsular lesions gave the appearance of “string of pearl.” Pure tone audiometry confirmed the left side sensorineural hearing impairment. Fluorescein angiogram exhibited multiple branch retinal arterial occlusion. Patient was treated successfully with steroids and intravenous immunoglobulin.
Conclusion: We report the first case report of Susac syndrome in Sri Lanka. It is rare to get all 3 clinical components in the beginning of disease. Early and aggressive treatment can prevent permanent disability.
Keywords: Susac Syndrome; “Snowball” Lesions; “String of Pearl” Appearance; Retinocochleocerebral Vasculopathy; Arterial Wall Hyper Fluorescence
Citation: Chaminda Kumara. “Susac Syndrome with Typical Clinical Triad: First Case Report in Sri Lanka". Acta Acta Scientific Neurology 6.1 (2023): 36-40.
Copyright: © 2023 Chaminda Kumara. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.