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Vergunova I.Yu.

Novosibirsk Regional Clinical Hospital, Novosibirsk, Russia

Malkova N.A.

Novosibirskiĭ okruzhnoĭ tsentr rasseiannogo skleroza

Korobko D.S.

Oblastnoĭ tsentr rasseiannogo skleroza, Gosudarstvennaia novosibirskaia oblastnaia klinicheskaia bol'nitsa

Susac syndrome, review and clinical case

Authors:

Vergunova I.Yu., Malkova N.A., Korobko D.S.

More about the authors

Journal: S.S. Korsakov Journal of Neurology and Psychiatry. 2019;119(2‑2): 52‑57

DOI: 10.17116/jnevro20191192252

Read: 14123 times

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Table of contents

SUSAC SYNDROME, REVIEW AND CLINICAL CASE
SUSAC SYNDROME, REVIEW AND CLINICAL CASE

To cite this article:

Vergunova IYu, Malkova NA, Korobko DS. Susac syndrome, review and clinical case. S.S. Korsakov Journal of Neurology and Psychiatry. 2019;119(2‑2):52‑57. (In Russ.)
https://doi.org/10.17116/jnevro20191192252

Подписка 2026 Журнал неврологии и психиатрии им. С.С. Корсакова. Спецвыпуски (S.S. Korsakov Journal of Neurology and Psychiatry (special issues))
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Abstract:

Susac syndrome (SS) is an extremely rare neurological disorder characterized by symptoms of a clinical triad: encephalopathy, retinopathy and hearing loss. These problems arise from the microangiopathy of blood vessels that supply blood to the brain, the retina and the cochlea. SS is more common in young women. The authors present a literature review on the diagnosis and treatment of SS and the clinical observation of patients with classic signs of the disease triad.

Keywords:

Susac syndrome
diagnosis
treatment
prognosis

Authors:

Vergunova I.Yu.

Novosibirsk Regional Clinical Hospital, Novosibirsk, Russia

Malkova N.A.

Novosibirskiĭ okruzhnoĭ tsentr rasseiannogo skleroza

Korobko D.S.

Oblastnoĭ tsentr rasseiannogo skleroza, Gosudarstvennaia novosibirskaia oblastnaia klinicheskaia bol'nitsa

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References:

  1. Rennebohm RM, Egan RA, Susac JO. Treatment of Susac’s syndrome. Curr Treat Options Neurol. 2008;10(1):67-74.
  2. Jarius S, Kleffner I, Dörr JM, Sastre-Garriga J, Illes Z, Eggenberger E, Chalk C, Ringelstein M, Aktas O, Montalban X, Fechner K, Sto¨cker W, Ringelstein EB, Paul F, Wildemann B. Clinical, paraclinical and serological findings in Susac syndrome: an international multicenter study. J Neuroinflammation. 2014;11(1):46-50.
  3. Dörr J, Krautwald S, Wildemann B, Jarius S, Ringelstein M, Duning T, Aktas O, Ringestein EB, Paul F, Kleffner I Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 2013;9(6):307-316.
  4. Vodopivec I, Sashank P. MD Short Follow-up Bias Confounds Estimates of the «Typical» Clinical Course of Susac Syndrome. J Neurophthalmol. 2016;16:23-28.
  5. Martinet N, Fardeau C, Adam R, Bodaghi B, Papo T, Piette JC, Lehoang P. Fluorescein and indocyanine green angiographies in Susac syndrome. Retina. 2007;27:1238-1242.
  6. Bucciarelli S, Cervera R, Martinez M, Latorre X, Font J. Susac’s syndrome or catastrophic antiphospholipid syndrome. Lupus. 2004;3:607-608.
  7. Demir MK. Case 142: Susac syndrome. Radiology. 2009;250:598-602.
  8. Prakash G, Jain S, Gupta M, Nathi T. Susac’ssyndrome: first from India and youngest in the world. Indian J Ophthalmol. 2013;61(12):772-773.
  9. Turc G, Monnet D, Dupin N, Beuvon F, Guiraud V, Ben Amor M, Touzé E. Skin involvement in Susac’s syndrome. J Neurol Sci. 2011;305:152-155.
  10. Susac JO, Murtagh FR, Egan RA, Berger JR, Bakshi R, Lincoff N, Gean AD, Galetta SL, Fox RJ, Costello FE, Lee AG, Clark J, Layzer RB, Daroff RB. MRI findings in Susac’s syndrome. Neurology. 2003;61(12):1783-1787.
  11. Hua le H, Donlon SL, Okuda DT. A case of Susac syndrome with cervical spinal cord involvement on MRI. J Neurol Sci. 2014;337(1-2):228-231.
  12. Mario García-Carrasco, Claudia Mendoza-Pinto, Ricard Cervera Diagnosis and classification of Susac syndrome. Autoimmunity Reviews. 2014;13:349-359.
  13. Rennebohm RM, Asdaghi N, Srivastava S, Gertner E. Guidelines for treatment of Susac syndrome — An update. Int J Stroke. 2018;1:1747493017751737. https://doi.org/10.1177/1747493017751737
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