Susac Syndrome: Case Report

Síndrome de Susac: reporte de caso

Objective. To describe the case of a 23 year-old female patient disoriented in space and time, with an oppressive left-sided temporal headache, associated with ataxia, and bradypsychia. Methods. A possible multiple sclerosis associated to the clinical and paraclinical tests was suspected at the beginning. An acute disseminated encephalomyelitis was suspected and treated with methylprednisolone pulses and patient was discharged. Within a month, she required again due to exacerbation of neurological symptoms given by a loss of strength in upper and lower limbs, dysarthria, wetting, and drowsiness. Devic’s disease was diagnosed initially. Because response to corticosteroids was not the expected, it was decided to treat with plasmapheresis; in addition, new tests were requested to rule out the suspected disease. Results. Treatment was focused on a possible diagnosis of Susac’s syndrome with an arteriography retinal test and potential auditory evoked.

---

Article visits 196 | PDF visits 119

---

Downloads

Download data is not yet available.

1. Susac JO, Hardman JM, Selhorst JB. Microangiopathy of the brain and retina. Neurology 2003; 29:313–316.
2. Schwitter J, Agosti R, Ott P, Kalman A, Waespe W. Small infarctions of cochlear, retinal, and encephalic tissue in young women. Stroke. 1992;23:903-907.
3. Tai Hung Do, Christiane Fisch, and François Evoy. Susac Syndrome: Report of Four Cases and Review of the Literature. AJNR 2004; 25: 382-388.
4. Álvarez Pinzón Andrés Mauricio, Neuromielitis óptica. Revista Salud Bosque 2012;2:35-45.
5. Han Joo Cho, Chul Gu Kim, Sung Won Cho, and Jong Woo Kim. A Case of Susac Syndrome. Korean J Ophthalmol. 2013; 27: 381–383.
6. Magro, Cynthia MMD; Poe, Jonathan C, PhD; Susac, John O, MD.American. Susac Syndrome: An Organ-Specific Autoimmune Endotheliopathy Syndrome Associated With Anti-Endothelial Cel Antibodie. Journal of Clinical Pathology136.6 (Dec 2011): 903-12.
7. Department of Ophthalmology Luton and Dunstable NHS Trust Luton, UK. V Sandhya and N Anand. Susac Syndrome. Eye 2002;16, 788–790.
8. McLeod DS, Ying HS, McLeod CA. Retinal and optic nerve head pathology in Susac’s syndrome. Ophthalmology. 2011 March; 118: 548–552.
9. Thomas C, Mini S, Thomas SV. Rescue plasmapheresis in a cas of severe steroid unresponsive devic`s nuromyelitis optica. Ann Indian Acad Neurol 2006; 9:42-45.
10. Lammouchi, Turkia M. Bouker, Samira M. Grira, Mounir T. Benammou, Sofien A. Susac’s syndrome. Saudi Med J 2004;25: 222-224.
11. Bienfang, Don C/DC; McKenna, Michael J/MJ; Case records of the Massachusetts General Hospital. Case 24-2011. A 36-year-old man with headache, memory loss, and confusion. N Engl J Med 2011; 365:549-559.
12. Prakash, Gunjan; Jain, Shephali;; Nathi, Tirupat. Susac’s syndrome: First from India and youngest in the world. Indian Journal of Ophthalmology61.12 (Dec 2013): 772-773.
13. Kleffner I, Duning T, Lohmann H, et al. A brief review of Susac syndrome. J Neurol Sci 2012;322:35–40.
14. Ortiz Alberto, Roverano Susana, Paira Sergio. Síndrome Susac: presentación de un caso y revisión de la literatura. Revista Argentina de Reumatología 2010;21:29-32.
15. Dörr J, Krautwald S, Wildemann B, et al. Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol 2013; 9:307–316.
16. T.A. Hardy, R.J. Garsia, G.M. Halmagyi, S.J. Lewis, B. Harrisberg, M.J. Fulham, et al. Tumour necrosis factor (TNF) inhibitor therapy in Susac’s síndrome. J. Neurol. Sci., 302 (2011), pp. 126–128.