To illustrate the classic brain MRI changes in patients with Susac Syndrome. To describe the spectrum of MRI abnormalities in this disease. To increase awareness about this rare disease and to emphasize the importance of brain MRI in the diagnosis.

Susac syndrome (SS) is a rare autoimmune disease that typically affects 20-40-year-old females. The syndrome was first described by John O. Susac in 1979 [1] and since then it has been recognized more often as the diagnostic tools improve, although the real incidence is unknown. The underlying pathophysiology is microangiopathy with occlusion of small vessels in the brain, retina, and cochlea, giving the classic clinical triad with CNSdysfunction, visual disturbance, and hearing loss. This disease has also been called “small infarctions of cochlear, retinal and...

We selected all the 8 cases of confirmed Susac syndrome at our institution and retrospectively reviewed the brain MRI of each patient. Six patients were female and the median age at diagnosis was 33 years old (ranging from 28 to 55 years of age). Not all of them had the clinical triad from the onset of symptoms and in two of them, the diagnosis was suggested on MRI. All patients were studied with at least one MRI study at our institution (either 1.5T or 3T...

Susac Syndrome is a rare disease and a better understanding of the disease is of clinical relevance. In our experience, MRI is one of the keystones to diagnose Susac Syndrome. White matter lesions with high signal in T2-weighted sequences, namely in the corpus callosum are the most common finding. ‘Snowball lesions’ in corpus callosum are typical but not demanding for the diagnosis. Correct diagnose is essential once there are therapies that modify the disease course and improve the quality of life.

C. M. Pinto; Porto/PT - nothing to disclose C. Ramos; Porto/PT - nothing to disclose

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