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Headache in Susac’s Syndrome

  • Secondary Headache (M Robbins, Section Editor)
  • Published:
Current Pain and Headache Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

Provide an overview of the current diagnosis, pathophysiology, and treatment of Susac’s syndrome (SuS), with special emphasis on summarizing what is currently known about headache as a symptom of disease activity.

Recent Findings

The most recent literature in SuS has focused on furthering the understanding of the underlying pathology and efficacy of treatments for SuS. The importance of early recognition to facilitate timely treatment and avoid long-term disability has been highlighted. Headache, the most common symptom experienced by patients with SuS, can occur up to 6 months in advance of other symptoms, and exacerbations of headache can herald increased disease activity.

Summary

Susac’s syndrome (SuS) is a rare disorder classically characterized by triad of encephalopathy, branch retinal artery occlusion (BRAO), and sensory neuronal hearing loss (SNHL). The full triad is uncommon at initial presentation, which can confound efforts to make timely diagnosis and treatment decisions. Headache is the most common symptom in SuS, is often an early feature, and can help separate SuS from other diagnoses in the differential. However, the features and management of the headache associated with SuS have not been systematically defined in the literature.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Susac JO, Egan RA, Rennebohm RM, Lubow M. Susac’s syndrome: 1975-2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci. 2007;257:270–2.

    Article  Google Scholar 

  2. Rennebohm RM, Susac JO, Egan RA, Daroff RB. Susac’s syndrome – update. J Neurol Sci. 2010;299:86–91.

    Article  Google Scholar 

  3. Gross CC, Meyer C, Bhatia U, Yshii L, Kleffner I, Bauer J, et al. CD8+ T cell-mediated endotheliopathy is a targetable mechanism of neuro-inflammation in Susac syndrome. Nat Commun. 2019;10:5779.

    Article  CAS  Google Scholar 

  4. Vodopivec I, Prasad S. Short follow-up bias confounds estimates of the ‘typical’ clinical course of Susac syndrome. J Neuroophthalmol. 2017;37:149–53.

    Article  Google Scholar 

  5. Seifert-Held T, Langner-Wegscheider BJ, Komposch M, Simschitz P, Franta C, Teuchner B, et al. Susac’s syndrome: clinical course and epidemiology in a Central European population. Int J Neurosci. 2017;127(9):776–80.

    Article  Google Scholar 

  6. Heng LZ, Bailey C, Lee R, Dick A, Ross A. A review and update on the ophthalmic implications of Susac syndrome. Surv Ophthalmol. 2019;64:477–85.

    Article  Google Scholar 

  7. Dörr J, Krautwald S, Wildemann B, Jarius S, Ringelstein M, Duning T, et al. Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 2013;9:307–16.

    Article  Google Scholar 

  8. Marrodan M, Correale J, Alessandro L, Amaya M, Fracaro ME, Köhler AA, et al. Susac syndrome: a differential diagnosis of white matter lesions. Mult Scler Relat Disord. 2017;15:42–6.

    Article  Google Scholar 

  9. Rennebohm RM, Susac JO. Treatment of Susac’s syndrome. J Neurol Sci. 2007;257:215–20.

    Article  Google Scholar 

  10. Vodopivec I, Prasad S. Treatment of Susac syndrome. Curr Treat Options Neurol. 2016;18:3.

    Article  Google Scholar 

  11. Rennebohm RM, Asdaghi N, Srivastava S, Gertner E. Guidelines for treatment of Susac syndrome – an update. Int J Stroke. 2018;0(0):1–11 This article has the most up to date and a comprehensive review of treatment options and the available evidence for Susac syndrome. The authors have published several prior articles on the topic.

    Google Scholar 

  12. Kleffner I, Dörr J, Ringelstein M, Gross C, Böckenfeld Y, Schwindt W, et al. Diagnostic criteria for Susac syndrome. J Neurol Neurosurg Psychiatry. 2016;87(12):1287–95 This article first published diagnostic criteria for Susac syndrome, validated based on both an unpublished cohort and the above published cohort (Dorr). The authors belong to the European Susac Consortium (EuSaC) and the EuSaC study group.

    Article  Google Scholar 

  13. Egan RA. Diagnostic criteria and treatment algorithm for Susac syndrome. J Neuroophthalmol. 2019;39(1):60–7 This article builds on the diagnostic criteria published by Kleffner et al. [ref 13], suggesting that there are 2 findings that allow for the diagnosis of definite Susac syndrome even when the clinical triad is not fully present, which might allow for earlier diagnosis and therefore earlier treatment.

    Article  Google Scholar 

  14. Vishnevskia-Dai V, et al. Susac syndrome: clinical characteristics, clinical classification, and long-term prognosis. Medicine. 2016;95(44):e5223.

    Article  Google Scholar 

  15. Headache Classification Committee of the International Headache Society (IHS). The international classification of headache disorders, 3rd edition. Cephalgia. 2018;38(1):1–211.

    Article  Google Scholar 

  16. Kefi S, et al. Headache in a young woman: do not forget Susac's syndrome. Reumatismo. 2017;69(3):122–5.

    Article  CAS  Google Scholar 

  17. Vodopivec I, Venna N, Rizzo JF III, Prasad S. Clinical features, diagnostic findings and treatment of Susac syndrome: a case series. J Neurol Sci. 2015;357:50–7.

    Article  Google Scholar 

  18. Sauma J, Rivera D, Wu A, Donate-Lopez J, Gallego-Pinazo R, et al. Susac’s syndrome: an update. Br J Ophthalmol. 2020;0:1–6.

    Google Scholar 

  19. Triplett JD, et al. Immune-mediated conditions affecting the brain, eye and ear (BEE syndromes). J Neurol Neurosurg Psychiatry. 2019;90:822–94.

    Article  Google Scholar 

  20. Obelienienė D, Macaitytė R, Balnytė R, Pėstininkaitė R, Gleiznienė R, Balčiūnienė J. Characteristics of headache in relation to the manifestation of Susac syndrome. Medicina. 2017;53:420–5.

    Article  Google Scholar 

  21. Kowacs F, Ferreira Gomes M, Pigozzo T, Rodrigues Palmeira AL, Muratt Carpenedo C, Castro Ribeiro M. Migraine-like headache as presentation symptom in Susac syndrome. Headache. 2016;56(10):1667–9.

    Article  Google Scholar 

  22. Milbratz GH, Marquardt FA, Guimarães Neto HP, Marquardt DA, de Souza ESMV. Retinal vasculitis in Susac syndrome: case report. Arq Bras Oftalmol. 2009;72(3):397–9.

    Article  Google Scholar 

  23. Ramey LN, Lopez E, Young T. Rehabilitation of Susac syndrome. Am J Phys Med Rehabil. 2016;95(4):e48–52.

    Article  Google Scholar 

  24. Chawla A, Sathasivam S, Nayar R, Doran M. Susac syndrome in a patient with hepatitis C. J Neuroophthalmol. 2007;27:55–6.

    Article  Google Scholar 

  25. Rivadeneira-Sotelo C, et al. Susac syndrome: the first case report in Peru. Síndrome de Susac: primer reporte de caso en el Perú. Medwave. 2017;17(7):e7033.

    Article  Google Scholar 

  26. Mallam B, Damato EM, Scolding NJ, Bailey C. Serial retinal fluorescein angiography and immune therapy in Susac’s syndrome. J Neurol Sci. 2009;285:230–4.

    Article  Google Scholar 

  27. Vetvik KG. Epidemiology of migraine in men and women. In: Maassen van den Brink A, MacGregor E, editors. Gender and migraine. Headache. Switzerland: Springer, Cham; 2019. p. 1–15.

    Google Scholar 

  28. Agamanolis DP, Prayson RA, Asdaghi N, Gultekin SH, Bigley K, Rennebohm RM. Brain microvascular pathology in Susac syndrome: an electron microscopic study of five cases. Ultrastruct Pathol. 2019;43(3):229–36.

    Article  Google Scholar 

  29. Agamanolis DP, Klonk C, Bigley K, Rennebohm RM. Neuropathological findings in Susac syndrome: an autopsy report. J Neuropathol Exp Neurol. 2019;78(6):515–9.

    Article  Google Scholar 

  30. Jarius S, et al. Clinical, paraclinical and serological findings in Susac syndrome: an international multicenter study. J Neuroinflammation. 2014;11(46):1–11.

    Google Scholar 

  31. Gomez-Figueroa E, Garcia-Trejo S, Garcia-Santos RA, Quiñones-Pesqueira G, Calleja-Castillo JM. Exquisite response to intravenous immunoglobulin in Susac syndrome during pregnancy. eNeurologicalSci. 2018;10:1–4.

    Article  Google Scholar 

  32. Marrodan M, Acosta JN, Alessandro L, Fernandez VC, Carnero Contentti E, Arakaki N, et al. Clinical and imaging features distinguishing Susac syndrome from primary angiitis of the central nervous system. J Neurol Sci. 2018;395:29–34.

    Article  CAS  Google Scholar 

  33. Engisch R, Titelbaum DS, Chilver-Stainer L, Kellner-Weldon F. Susac’s syndrome: leptomeningeal enhancement on 3D FLAIR MRI. Mult Scler. 2016;22(7):972–4.

    Article  CAS  Google Scholar 

  34. Susac JO, Murtagh FR, Egan RA, Berger JR, Bakshi R, Lincoff N, et al. MRI findings in Susac’s syndrome. Neurology. 2003;61(12):1783–7.

    Article  CAS  Google Scholar 

  35. Fernando SL, Boyle T, Smith A, Parratt JD. The successful use of infliximab in a relapsing case of Susac’s syndrome. Case Rep Neurol Med. 2020;2020:9317232. https://doi.org/10.1155/2020/9317232.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Ramos-Ruperto L, Martinez-Sanchez N, Bartha-Rasero JL, Robles-Marhuenda A. Susac syndrome and pregnancy: a relationship to clarify. About two cases and review of the literature. J Matern Fetal Neonatal Med. 2020:1–6. https://doi.org/10.1080/14767058.2020.1712712.

  37. Bhattacharjee S, Janjua F, Elkider M, Sweeney B. An unusual cause of chronic headache in a young man. Headache. 2016;56:370–1.

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Medicine,Division of Neurology, Dalhousie University,Halifax Infirmary, 1796 Summer Street, Halifax, NS, B3H 3A7, Canada

    Jessica A. Dawe

  2. Department of Neurology, Dartmouth-Hitchcock Medical Center and Geisel School of Medicine at Dartmouth, One Medical Center Drive, Lebanon, NH, 03766, USA

    A. Laine Green

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Correspondence to A. Laine Green.

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Conflict of Interest

Jessica A. Dawe declares no conflicts of interests. A. Laine Green declares no conflicts of interest with respect to the submitted work. A. Laine Green reports other from Allergan Canada, other from Novartis, other from Eli Lilly, outside the submitted work.

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Dawe, J.A., Green, A.L. Headache in Susac’s Syndrome. Curr Pain Headache Rep 25, 25 (2021). https://doi.org/10.1007/s11916-021-00939-2

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