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The following is a summary of “Cognitive outcomes in Susac syndrome: A 2-year neuropsychological follow-up study,” published in the February 2024 issue of Neurology by Vrekheme et al.

Susac syndrome (SuS) is an uncommon autoimmune disorder featuring a triad of branch retinal artery occlusion, sensorineural hearing loss, and encephalopathy, with a limited understanding of its cognitive deficits’ prevalence, nature, and progression.

Researchers started a retrospective study to elucidate the long-term neuropsychological outcomes of individuals affected by SuS.

They recruited thirteen patients diagnosed with SuS (mean [SD] age: 39.5 [11.1] years) from Ghent University Hospital under the care of their neurologists. Each patient underwent comprehensive neuropsychological assessments for cognitive functioning and emotional well-being at baseline and after a 2-year interval. Follow-up evaluations were conducted with 11 patients (mean [SD] age: 42.2 [11.5] years).

The result showed that, at a group level, patients exhibited normal neuropsychological test outcomes at baseline and during follow-up assessments. Over time, significant enhancements were observed in information processing speed, verbal recognition, and semantic and phonological fluency. At baseline, individual test results displayed interindividual variability, with attention, executive functioning, and language being the most affected domains, all showing improvement after 2 years. Patients reported significantly reduced mental and physical well-being levels, persisting from baseline to follow-up assessments.

Investigators concluded that SuS patients may not show a significant group-level cognitive decline, but individual evaluations and psycho-emotional support remain crucial due to variability and high stress.

Source: onlinelibrary.wiley.com/doi/full/10.1111/ene.16186