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.

Alemtuzumab in multiple sclerosis: A retrospective analysis of occult hemorrhagic magnetic resonance imaging lesions and risk factors.

BACKGROUND AND PURPOSE: Alemtuzumab, a monoclonal CD52 antibody, is a high-efficacy disease-modifying-therapy in relapsing-remitting multiple sclerosis (RRMS). Recently, intracerebral hemorrhage (ICH) was reported as a possible treatment-related adverse event. Arterial hypertension during infusion was suggested as a potential cause, although platelet or endothelial dysfunction may also contribute. This study aimed to screen for occult hemorrhagic cerebral lesions after alemtuzumab treatment and to further elucidate risk factors. METHODS: We included 30 RRMS patients who received alemtuzumab treatment at Ghent University Hospital or Sint-Jan Bruges Hospital. Retrospective data concerning vital signs, adverse effects and thrombocyte levels during treatment were collected. The occurrence of occult intracranial hemorrhagic lesions was assessed by magnetic resonance imaging with susceptibility-weighted imaging (SWI). RESULTS: The mean (standard deviation [SD]) systolic blood pressure (SBP) during the morning, afternoon and evening was 120 (3.38) mmHg during first administration and 114 (4.40) mmHg during second administration (N = 13). There was no significant increase in SBP when comparing morning, afternoon and evening per day, nor was there a significant difference in daily mean SBP between consecutive administration days. Thrombocyte count during treatment cycles ranged between 107 × 109 /L and 398 × 109 /L, with a mean (SD) absolute reduction of 59.3 × 109 /L (50.65) or a mean (SD) relative reduction of 25.0 (12.84)% (N = 20). No patient had ICH, nor did SWI show any cerebral microbleeds or other hemorrhagic lesions post-treatment (N = 23). CONCLUSIONS: In our patient population, alemtuzumab treatment was not associated with arterial hypertension, ICH or occult microbleeds. Possible differences in administration regimen (ambulatory vs. in-hospital setting) and patient population (cardiovascular risk) might explain an increased risk in different populations.

Microbleed patterns in critical illness and COVID-19.

BACKGROUND: Cerebral microbleeds are increasingly reported in critical ill patients with respiratory failure in need of mechanical ventilation and/or extracorporeal membrane oxygenation (ECMO). Typically, these critical illness-associated microbleeds involve the juxtacortical white matter and corpus callosum. Recently, this pattern was reported in patients with respiratory failure, suffering from COVID-19. MATERIALS AND METHODS: In this retrospective single-center study, we listed patients from March 11, 2020 to September 2, 2020, with laboratory-confirmed COVID-19, critical illness and cerebral microbleeds. Literature research was conducted through a methodical search on Pubmed databases on critical illness-associated microbleeds and cerebral microbleeds described in patients with COVID-19. RESULTS AND DISCUSSION: On 279 COVID-19 admissions, two cases of cerebral microbleeds were detected in critical ill patients with respiratory failure due to COVID-19. Based on review of existing literature critical illness-associated microbleeds tend to predominate in subcortical white matter and corpus callosum. Cerebral microbleeds in patients with COVID-19 tend to follow similar patterns as reported in critical illness-associated microbleeds. Hence, one patient with typical critical illness-associated microbleeds and COVID-19 is reported. However, a new pattern of widespread cortico-juxtacortical microbleeds, predominantly in the anterior vascular territory with relative sparing of deep gray matter, corpus callosum and infratentorial structures is documented in a second case. The possible etiologies of these microbleeds include hypoxia, hemorrhagic diathesis, brain endothelial erythrophagocytosis and/or cytokinopathies. An association with COVID-19 remains to be determined. CONCLUSION: Further systematic investigation of microbleed patterns in patients with neurological impairment and COVID-19 is necessary.

Vestibular schwannoma: natural growth and possible predictive factors.

Background: Prediction of vestibular schwannoma (VS) growth would allow for a more differentiated follow-up protocol. Objectives: The natural course of a VS and predictive factors of growth are investigated. Methods: Sixty-two sporadic VS cases diagnosed between 2003 and 2015 were included in this retrospective cohort study. After initial surveillance, active therapy was initiated in 31/62 patients. Regular magnetic resonance images (MRIs) were performed. Two mm/year linear difference was the cut-off value for significant growth. The STROBE guidelines have been implemented. Results: Growth of the tumor was detected in 56% of patients and mainly observed in the first three years of follow-up. Tumor size remained stable in 34% and decreased in 10% of patients. No baseline information, symptom, or sign was found to be predictive for growth. Conclusions: In 56% of the initially conservatively managed VSs growth was observed and active treatment was initiated. Eighty-seven percent of the growing VSs were identified during the first three years of follow-up. The initially larger VSs seemed to grow faster and needed active treatment earlier during follow-up. No predicting factors for growth identified from the literature could be confirmed in the present study. Serial MRI remains the appropriate method to detect tumor growth.