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

Abstract

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

References

1. Susac JO., et al. “Susac's syndrome: 1975-2005 microangiopathy/autoimmune endotheliopathy”. Journal of the Neurological Sciences 1-2 (2007): 270-272.
2. Susac JO., et al. “Microangiopathy of the brain and retina”. Neurology 3 (1997): 313-316.
3. Susac JO. “Susac's syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women”. Neurology4 (1994): 591-593.
4. Vodopivec I and Prasad S. “Treatment of Susac syndrome”. Current Treatment Options in Neurology 1 (216): 3.
5. Seifert-Held T., et al. “Susac's syndrome: clinical course and epidemiology in a central European population”. International Journal of Neuroscience 9 (2017): 776-780.
6. Vishnevskia-Dai V., et al. “Susac syndrome: clinical characteristics, clinical classification, and long-term prognosis”. Medicine (Baltimore)43 (2016): e5223.
7. Kleffner I., et al. “Diagnostic criteria for Susac syndrome”. Journal of Neurology, Neurosurgery and Psychiatry 12 (2016): 1287-1295.
8. Brandt AU., et al. “RETINAL LESION EVOLUTION IN SUSAC SYNDROME”. Retina2 (2016): 366-374.
9. Ringelstein M., et al. “Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography”. Neurology 7 (2015): 610-618.
10. Susac JO., et al. “MRI findings in Susac's syndrome”. Neurology12 (2015): 1783-1787.
11. Egan RA., et al. “Gass plaques and fluorescein leakage in Susac syndrome”. Journal of the Neurological Sciences 1-2 (2010): 97-100.
12. Jarius S., et al. “Anti-endothelial serum antibodies in a patient with Susac's syndrome”. Journal of the Neurological Sciences 1-2 (2009): 259-261.
13. Magro CM., et al. “Susac syndrome: an organ-specific autoimmune endotheliopathy syndrome associated with anti-endothelial cell antibodies”. American Journal of Clinical Pathology 6 (2011): 903-912.
14. Winterholler M., et al. “The Susac syndrome-a retinocochleocerebral angiopathy [German]”. Fortschritte der Neurologie-Psychiatrie 68 (2000): 475-481.
15. Macleod DS., et al. “Retinal and optic nerve head pathology in Susac Syndrome”. Ophthalmology3 (2011): 548-582.
16. Patel VA., et al. “Otologic manifestations of Susac Syndrome”. ACTA Otorhinolaryngologica Italica 6 (2018): 544-553.
17. Wilf-Yakoni A., et al. “Increased incidence of Susac Syndrome”. BMC Neurology (2020): 332.

Citation

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

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.