The Classic Triad of Susac Syndrome

A 31-year-old female, right handed, previously healthy, presents with progressive headaches, intermittent visual disturbance, and mild confusion. Examination revealed a superior temporal field defect in the right eye. MRI brain was obtained (sagittal non-contrast T1-weighted image is shown). Lumbar puncture showed 18 white blood cells (87% lymphocytes), protein 196.5 mg/dL, and glucose 60 mg/dL. 

This patient had several characteristic features of Susac syndrome, a rare immune-mediated vasculopathy that mainly affects young women but can also affect men. The classic triad of Susac syndrome – encephalopathy, branch retinal artery occlusion (BRAO), and hearing loss – is present in only a minority of patients. The BRAO was responsible for our patient’s field defect. “Snowball” lesions in the corpus callosum, as shown here, are a characteristic finding. Prompt immunosuppressive therapy is indicated.


Further Reading

  1. Clinical Features, Diagnostic Findings, and Treatment of Susac Syndrome: A Case Series
  2. Figure 1 Medical Case
  3. Susac Syndrome: Clinical Characteristics, Clinical Classification, and Long-Term Prognosis

References

  1. Kleffner I, Dörr J, Ringelstein M, Gross CC, Böckenfeld Y, Schwindt W, Sundermann B, Lohmann H, Wersching H, Promesberger J, von Königsmarck N, Alex A, Guthoff R, Frijns CJ, Kappelle LJ, Jarius S, Wildemann B, Aktas O, Paul F, Wiendl H, Duning T; European Susac Consortium (EuSaC). Diagnostic criteria for Susac syndrome. J Neurol Neurosurg Psychiatry. 2016 Dec;87(12):1287-1295. doi: 10.1136/jnnp-2016-314295. Epub 2016 Oct 25. PMID: 28103199.
  2. Pereira S, Vieira B, Maio T, Moreira J, Sampaio F. Susac’s Syndrome: An Updated Review. Neuroophthalmology. 2020 May 1;44(6):355-360. doi: 10.1080/01658107.2020.1748062. PMID: 33408428; PMCID: PMC7746245.

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