Cognitive outcomes in Susac syndrome: A 2-year neuropsychological follow-up study.

BACKGROUND AND PURPOSE: Susac syndrome (SuS) is a rare, autoimmune, neurological disease characterized by a clinical triad of branch retinal artery occlusion, sensorineural hearing loss and encephalopathy. Neuropsychological functioning in SuS is little researched and the prevalence, nature, and evolution over time of cognitive deficits in SuS remain unclear. This study aimed to better understand the long-term neuropsychological outcomes of patients with SuS. METHODS: Thirteen patients with SuS (mean [SD] age 39.5 [11.1] years) were enrolled at the Ghent University Hospital by their treating neurologist. The cognitive functioning and emotional well-being of each patient was evaluated by means of a thorough neuropsychological test battery at baseline and after 2 years. Follow-up testing after 2 years was performed in 11 patients (mean [SD] age 42.2 [11.5] years). RESULTS: Patients showed normal neuropsychological test results at a group level, both at baseline and follow-up testing. Significant improvements over time were found for information processing speed, verbal recognition, and semantic and phonological fluency. Individual test results showed interindividual variability at baseline, with most impairments being in attention, executive functioning and language, which improved after a 2-year period. In addition, patients reported significantly lower mental and physical well-being, both at baseline and follow-up testing. CONCLUSIONS: Our results suggest that neuropsychological dysfunction in SuS is limited at a group level and improves over time. Nonetheless, individual test results reveal interindividual variability, making cognitive screening essential. Furthermore, a high psycho-emotional burden of the disease was reported, for which screening and follow-up are necessary.

Atypical clinical and novel radiological findings in Susac syndrome: Experience from a large monocentric cohort.

Susac syndrome (SuS) is a rare immune-mediated endotheliopathy that affects the brain, retina and inner ear and is characterised by the variable clinical triad of encephalopathy, visual and vestibulocochlear dysfunction. Here, we present clinical and paraclinical data of 19 SuS patients followed at Ghent University Hospital and highlight some atypical clinical and novel radiological findings. Our findings suggest that spinal involvement expands the clinical phenotype of SuS. We further introduce dark blood sequences as a more sensitive technique to detect radiological disease activity in SuS. Our data add to the current understanding of the diagnosis, monitoring and treatment of SuS.

The interplay between emotion regulation, emotional well-being, and cognitive functioning in brain tumor patients and their caregivers: An exploratory study.

OBJECTIVE: Brain tumor patients may suffer from a range of health-impairing problems reducing their quality of life. To identify potential targets for interventions, we examined the influence of different emotion regulation strategies on affective and cognitive functioning as indices of quality of life in patients and their caregivers in the early phase of treatment. METHODS: To this end, we conducted an exploratory longitudinal study on a small cohort, measuring emotion regulation, emotional well-being, and cognitive functioning on the day before each patient's tumor resection (28 patients and 11 caregivers) and several months after neurosurgery (22 patients and 10 caregivers). RESULTS: Results showed that emotion regulation strategies are relatively stable from preoperative to postoperative assessment. Nevertheless, several associations between emotion regulation strategies and quality of life indices were evident after tumor resection. In particular, our results were largely in line with previous research findings in healthy and other patient populations, corroborating the adaptive character of cognitive reappraisal, whereas suppression and expression of emotions were related to reduced cognitive and affective functioning, respectively. CONCLUSIONS: Based on these results, we suggest that further intervention or qualitative studies explore whether therapeutic interventions directed toward mastery of cognitive reappraisal techniques and appropriate expression of emotions could lead to improved long-term adjustment among brain tumor patients and their caregivers.