Curr Treat Options Neurol. 2016 Jan;18(1):3. doi: 10.1007/s11940-015-0386-x.
Treatment of Susac Syndrome.
Current treatment options in neurology
Ivana Vodopivec, Sashank Prasad
Affiliations
Affiliations
- Massachusetts Eye and Ear Infirmary, Neuro-ophthalmology Service, 243 Charles Street, Boston, MA, USA. [email protected].
- Department of Neurology, Brigham and Women's Hospital, 75 Francis Street, Boston, MA, USA. [email protected].
- Harvard Medical School, Boston, MA, USA. [email protected].
- Department of Neurology, Brigham and Women's Hospital, 75 Francis Street, Boston, MA, USA. [email protected].
- Harvard Medical School, Boston, MA, USA. [email protected].
PMID: 26715396
DOI: 10.1007/s11940-015-0386-x
Abstract
OPINION STATEMENT: Susac syndrome is a microangiopathy of the brain, retina, and cochlea. Several lines of evidence support the concept that this disease is an acquired autoimmune disorder. Prospective, randomized, controlled studies of treatments are not available because the disease is rare. Furthermore, the average period of follow-up in reported cases is short, limiting a complete understanding of the natural history of the disease. Empirical treatment strategies are therefore based upon expert recommendations and anecdotal reports of response to various immunomodulators, and the appropriate duration of therapy is not known. In our opinion, the encephalopathic form of Susac syndrome should be treated early and aggressively to avoid cognitive dysfunction and disability. Induction therapy with pulse methylprednisolone frequently proves to be inadequate. Additional agents, including intravenous immunoglobulins, intravenous cyclophosphamide, or rituximab are often necessary to induce a sustained remission. Maintenance therapy with oral glucocorticoids combined with intravenous immunoglobulins, mycophenolate mofetil, methotrexate, azathioprine, cyclophosphamide, or rituximab is typically necessary to achieve a sustained remission. Aspirin may be used as an adjunctive agent, although evidence showing efficacy is scant. The response to treatment should be closely monitored by frequent clinical examinations, brain MRI, and fluorescein angiography. Once disease remission has been established, it appears prudent to continue maintenance treatment for at least two additional years, although the real long-term risk of future relapses remains unknown. Establishing a multicenter patient registry and biorepository is essential to study the pathogenesis of the disease, further define the duration of disease, identify reliable biomarkers that aid early diagnosis and assess risk of relapse, and develop effective disease-specific therapies.
Keywords: Antiplatelet agents; Immunosuppression; Susac syndrome; Treatment
References
- Pediatrics. 2004 Jul;114(1):276-81 - PubMed
- J Neurol Neurosurg Psychiatry. 2015 May;86(5):582-4 - PubMed
- Am J Clin Pathol. 2011 Dec;136(6):903-12 - PubMed
- Curr Treat Options Neurol. 2008 Jan;10(1):67-74 - PubMed
- Acta Ophthalmol Scand. 2005 Dec;83(6):757-8 - PubMed
- J Neurol Sci. 2007 Jun 15;257(1-2):270-2 - PubMed
- Ann Rheum Dis. 2015 Jan;74(1):204-10 - PubMed
- J Neurol Sci. 2007 Jun 15;257(1-2):215-20 - PubMed
- Pediatr Rheumatol Online J. 2008 Jan 29;6:3 - PubMed
- Medicine (Baltimore). 2007 Mar;86(2):93-102 - PubMed
- J Neuroophthalmol. 2013 Sep;33(3):260-2 - PubMed
- Ophthalmology. 2011 Mar;118(3):548-52 - PubMed
- Stroke. 1996 Jan;27(1):149-51 - PubMed
- Medicine (Baltimore). 1998 Jan;77(1):12-40 - PubMed
- Neurol Sci. 2012 Dec;33(6):1407-10 - PubMed
- Neurology. 2015 Aug 18;85(7):610-8 - PubMed
- Arch Ophthalmol. 2012 Jun;130(6):804-6 - PubMed
- Neurology. 1979 Mar;29(3):313-6 - PubMed
- Case Rep Neurol. 2014 May 21;6(2):171-5 - PubMed
- J Neurol Sci. 2009 Oct 15;285(1-2):230-4 - PubMed
- Acta Ophthalmol Scand. 1997 Feb;75(1):107-8 - PubMed
- J Neurol Sci. 2006 Dec 21;251(1-2):17-22 - PubMed
- Nat Rev Neurol. 2013 Jun;9(6):307-16 - PubMed
- Ophthalmology. 1986 Sep;93(9):1148-57 - PubMed
- Ann Rheum Dis. 2006 Dec;65(12):1545-50 - PubMed
- Otol Neurotol. 2011 Oct;32(8):1198-204 - PubMed
- Neurology. 1994 Apr;44(4):591-3 - PubMed
- Otol Neurotol. 2009 Jan;30(1):34-40 - PubMed
- Ophthalmology. 1998 Jun;105(6):1038-44 - PubMed
- Mult Scler. 2012 Nov;18(11):1592-9 - PubMed
- Stroke. 1992 Jun;23(6):903-7 - PubMed
- Ophthalmology. 1994 Mar;101(3):480-9 - PubMed
- J Neurol Sci. 2011 Mar 15;302(1-2):126-8 - PubMed
- J Neurol Sci. 2015 Oct 15;357(1-2):50-7 - PubMed
- Eur J Neurol. 2012 Jun;19(6):800-11 - PubMed
- Indian J Ophthalmol. 2013 Dec;61(12):772-3 - PubMed
- J Neuroinflammation. 2014 Mar 08;11:46 - PubMed
- Arthritis Rheum. 2004 Apr;50(4):1332-7 - PubMed
- Stroke. 1991 Jul;22(7):933-7 - PubMed
- Neurology. 2001 Jul 24;57(2):334-6 - PubMed
- AJNR Am J Neuroradiol. 2004 May;25(5):706-13 - PubMed
- Mayo Clin Proc. 2001 Sep;76(9):958-60 - PubMed
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