Clinical characteristics and treatment approaches in patients with Susac syndrome: a scoping review of cases.

BACKGROUND: Susac syndrome (SuS) is a rare disease characterized by encephalopathy, hearing impairment and visual disturbances. Immunosuppressive treatments are used based on the hypothesis that an autoimmune endotheliopathy drives the disease. However, a solid evidence-based treatment approach is lacking. The aim of this review is to provide an overview of patient characteristics, disease course and treatment patterns related to successful outcome that have been reported in literature since 2013. METHODS: Three reviewers conducted a systematic literature search in February 2022. The primary outcome was treatment used, derived from cases classified as probable or definite SuS, describing successful treatment outcome (i.e. no signs of disease activity for ≥ 1 month). Secondary outcomes were time-to-relapse and follow-up time. Published case reports and case series were included. Various clinical characteristics and treatment(s) were extracted and categorized into different phases of treatment. RESULTS: A total of 810 records was identified. 120 articles met inclusion criteria and 161 cases were extracted. Of these, 151 cases were classified as probable or definite SuS and included in the final analysis. Number of combinations of treatments used per treatment phase were: 6 empirically, 35 after confirmed diagnosis, 43 for maintenance treatment, 22 after relapse, 18 during maintenance post-relapse. Median follow-up time was 12.3 months (0.5; 120) and median time to relapse was 4 months (1; 120). CONCLUSION: This scoping review summarizes treatment approaches in patients with SuS, highlighting variability. International efforts to collect clinical, imaging and treatment data from patients with SuS in registries are needed, in order to provide less biased and long-term follow-up information on treatment response, predictors of relapse and patient outcomes. This may lead to more evidence-based therapeutic approaches.

Pacemaker guided screening for severe sleep apnea, a possible option for patients with atrial fibrillation: A systematic review and meta-analysis.

INTRODUCTION: Obstructive sleep apnea is often underdiagnosed in atrial fibrillation (AF) patients although it is an important risk factor. A systematic review and meta-analysis was performed to assess which techniques cardiac implantable electronic devices (CIED) and Holter monitors use to screen for sleep apnea (SA), and to evaluate if these are suitable for AF patients from a diagnostic accuracy perspective. METHODS: The search was conducted in accordance with the PRISMA-guidelines. PICO was defined as (P) patients with AF, (I) Holter monitors or CIED suitable for screening for SA, (C) overnight polysomnography (PSG), (O) positive screening with subsequent positive polysomnographic diagnosis of SA. Optimal index test cut-off points corresponding to reference test cut-off for severe SA (PSG-AHI ≥ 30) were compared. Meta-analysis was conducted for the diagnostic odds ratio (DOR), with forest plot and ROC-curve for summary DOR. RESULTS: A total of five prospective cohort studies (n = 192) were included in the systematic review of which four studies (n = 132) were included in the meta-analysis. All included studies use transthoracic impedance measurement as a screening parameter. No studies evaluating Holter monitors were included. The population consisted of patients indicated for pacemaker implantation. The summary DOR was 27.14 (8.83; 83.37), AUC was 0.8689 (0.6872; 0.9456) and Q* was 0.8390 (0.7482; 0.9013). CONCLUSION: At optimal pacemaker-cut-off, pacemaker-guided screening for severe SA in patients with AF can be an effective triage tool for clinical practice. Further studies with larger sample sizes are needed to strengthen the evidence for this conclusion.