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 |