Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2021 Sep 30;21(1):352.
doi: 10.1186/s12886-021-02101-3.

Manifestation of Susac syndrome during interferon beta-1a and glatiramer acetate treatment for misdiagnosed multiple sclerosis: a case report

Joanna Roskal-Wałek  1   2 , Michał Biskup  3 , Magdalena Dolecka-Ślusarczyk  4   5 , Anita Rosołowska  6 , Andrzej Jaroszyński  4 , Dominik Odrobina  4   7
Affiliations
Case Reports

Manifestation of Susac syndrome during interferon beta-1a and glatiramer acetate treatment for misdiagnosed multiple sclerosis: a case report

Joanna Roskal-Wałek et al. BMC Ophthalmol. .

Abstract

Background: Susac syndrome (SS) is characterized by the triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. However, the diagnosis of SS remains difficult because the clinical triad rarely occurs at disease onset, and symptom severity varies. SS symptoms often suggest other diseases, in particular multiple sclerosis (MS), which is more common. Misdiagnosing SS as MS may cause serious complications because MS drugs, such as interferon beta-1a, can worsen the course of SS. This case report confirms previous reports that the use of interferon beta-1a in the course of misdiagnosed MS may lead to exacerbation of SS. Moreover, our case report shows that glatiramer acetate may also exacerbate the course of SS. To the best of our knowledge, this is the first reported case of exacerbation of SS by glatiramer acetate.

Case presentation: We present a case report of a patient with a primary diagnosis of MS who developed symptoms of SS during interferon beta-1a treatment for MS; these symptoms were resolved after the discontinuation of the treatment. Upon initiation of glatiramer acetate treatment, the patient developed the full clinical triad of SS. The diagnosis of MS was excluded, and glatiramer acetate therapy was discontinued. The patient's neurological state improved only after the use of a combination of corticosteroids, intravenous immunoglobulins, and azathioprine.

Conclusions: The coincidence of SS signs and symptoms with treatment for MS, first with interferon beta-1a and then with glatiramer acetate, suggests that these drugs may influence the course of SS. This case report indicates that treatment with glatiramer acetate may modulate or even exacerbate the course of SS.

Keywords: Susac syndrome; case report; glatiramer acetate; interferon beta-1a; multiple sclerosis.

PubMed Disclaimer

Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
A First fluorescein angiography of the left eye: Late phase shows segmental arteriolar wall hyperfluorescence (arrows) and branch retinal artery occlusion (arrowheads). B Second fluorescein angiography of the left eye performed two weeks later shows reperfusion of the previously occluded retinal arterioles and resolution of the arteriolar wall hyperfluorescence. C Following fluorescein angiography of the right eye shows arteriolar wall hyperfluorescence (arrows) in a peripheral branch. D Following fluorescein angiography of the left eye shows a new branch retinal artery occlusion (arrowheads) and arteriolar wall hyperfluorescence (arrows)
Fig. 2
Fig. 2
Brain MRI (T2-weighted sequence, sagittal view) shows lesions (arrows) in the trunk and the genu of the corpus callosum consistent with the diagnosis of Susac syndrome. Some of the lesions have typical spoke-like appearance
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Buzzard KA, Reddel SW, Yiannikas C, Sean Riminton D, Barnett MH, Hardy TA. Distinguishing Susac’s syndrome from multiple sclerosis. J Neurol. 2015;262:1613–21. doi: 10.1007/s00415-014-7628-9. - DOI - PubMed
    1. Egan RA. Diagnostic Criteria and Treatment Algorithm for Susac Syndrome. J Neuroophthalmol. 2019;39:60–7. doi: 10.1097/WNO.0000000000000677. - DOI - PubMed
    1. 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. doi: 10.1038/s41467-019-13593-5. - DOI - PMC - PubMed
    1. 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;127:776–80. doi: 10.1080/00207454.2016.1254631. - DOI - PubMed
    1. Algahtani H, Shirah B, Amin M, Altarazi E, Almarzouki H. Susac syndrome misdiagnosed as multiple sclerosis with exacerbation by interferon beta therapy. Neuroradiol J. 2018;31:207–12. doi: 10.1177/1971400917712265. - DOI - PMC - PubMed

Publication types

MeSH terms