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. 2024 Jul 2;45(11):5181–5187. doi: 10.1007/s10072-024-07672-9

Table 1.

Proposed clinical and research priorities for addressing the neuropsychiatric features of Susac syndrome

Domain Goals Proposed strategies
Phenotyping

• Clarify the nature of neuropsychiatric features in Susac syndrome

• Identify those at risk of developing neuropsychiatric symptoms in Susac syndrome

• Development of a proposed standardised battery evaluating cognitive and psychiatric symptoms at presentation and longitudinally

• Development of a multisite, international registry

• Development of a minimum dataset for application by registry participants

Pathophysiology • Determine if neuropsychiatric symptoms in Susac syndrome are directly related to the disease process or secondary to the functional impacts of the illness and treatments • Identify reliable biomarkers (peripheral blood, neuroimaging, CSF, and histopathology), and investigate the relationship between clinical phenotypes and these biomarkers
Treatment

• Develop an evidence-based therapeutic guideline for Susac syndrome, which takes into consideration cognitive and psychiatric outcomes

• Understand the role of psychotropic medication in targeting neuropsychiatric symptoms in Susac syndrome

• Development of a multicentre, international patient registry which involves capture of a minimum data set on treatment and neuropsychiatric outcomes

• Investigate the correlation between the nature/dose/duration of treatment and cognitive and psychiatric outcomes

Long-term outcomes

• Better understand the cognitive and psychiatric prognosis of Susac syndrome

• Understand the predictors of long-term cognitive and psychiatric outcomes in Susac syndrome

• Inclusion of cognitive, psychiatric, and functional assessments longitudinally