Lesion probability map in cerebral vein thrombosis due to Behçet's disease.

AIM: Behçet's disease (BD) is a multisystemic inflammatory disease. Cerebral venous sinus thrombosis (CVST) is the second most common form of neuro-BD after parenchymal central nervous system involvement. The purpose of this study was to construct flow-void probability maps of patients with CVST with and without BD to visually illustrate the impacted cerebral venous sinuses, to compare the subgroups of patients, and investigate the effect of thrombus localization on clinical findings. METHODS: Seventeen patients with a diagnosis of BD-related CVST (CVST-BD) and 23 patients with a diagnosis of CVST related to other etiologies (CVST-O) were included. We collected data including gender, age at onset of BD and CVST, presenting symptoms, neurological findings, and the etiology. High-resolution magnetic resonance venographies obtained during CVST were used to mark and digitalize thrombosed areas. Thrombus probability and subtraction maps were created to reveal the differences between the subgroups. RESULTS: Remarkably, all patients with CVST-BD had thrombosis in the transverse sinus (TS). However, TS was affected in 73.9% of the CVST-O patients (17/17 in CVST-BD vs 17/23 in CVST-O, P = .03). Thrombosis developed mostly in the superior sagittal sinus (SSS) and TS in the CVST-O group (11/23, 47.8% and 17/23, 73.9%, respectively). The frequency of SSS thrombosis tended to be higher in the CVST-O (47.8% vs 23.5%, P = .19). CONCLUSION: Venous infarction and hemorrhage were less common in patients with CVST-BD. The only clinical symptom in most of the CSVT patients with BD was headache due to elevated intracranial pressure. TS thrombosis was more common in patients with BD.

The effect of cognitive performance on self-management behavior of multiple sclerosis patients.

BACKGROUND: Difficulties of self-management in people with MS (pwMS) is considered as one of the most important factors contributing to low rehabilitation efficacy, more severe long-term complications and increase in healthcare costs. Despite the emergence of research in the last decade documenting causes, types, and course of cognitive difficulties in MS disease subtypes, limited evidence is available in the literature for direct comparison of self-management and cognitive deficits. In this study we aimed to investigate the relationship between cognitive performance and self-management in pwMS. METHODS: PwMS who applied to neurology out-patient clinics of seven different centers were included into study. Multiple Sclerosis Self-Management Scale- Revised (MSSM-R) was used for the assessment of self-management behaviors and Multiple Sclerosis inventory cognition scale (MUSIC) was used for the assessment of cognitive performance and fatigue. RESULTS: In this study, 194 (144 female and 50 male) pwMS participated (mean age = 38.9 years). The course of the disease was RRMS in 173 patients and mean EDSS was 2.0. 68.5% of the participants were married, 32.5% were employed, and 57.2% had secondary education. The MSSM-R mean score of the study group was 42.6 ± 10.4 (1-81). There was a positive correlation between MSSM-R and MUSIC-cog scores (r = 0.21, p = 0.003). A hierarchical multiple regression revealed that income level (ß = 0.196, t = 2.692, p = 0.008) and cognitive performance (ß = 0.167, t = 2.063, p = 0.041) together with control variables (gender, age, educational status, employment status, duration of disease, EDSS and fatigue) explained 5.5% of the variance in self-management. CONCLUSION: Cognitive performance is a predictor of self-management in pwMS. Better self-management behavior is also related with employment and income level in pwMS. Studies evaluating patients' cognitive abilities and evaluating the effectiveness of adapted self-management training programs are needed.

Autoimmune Encephalitis After Treatment of Hodgkin's Lymphoma with the Immune Checkpoint Inhibitor Nivolumab.

In recent years, by the usage of new immune therapeutic agents for cancer treatment, the neurologic adverse events began to be seen more frequently. Nivolumab, one of the immune checkpoint inhibitor, is a human IgG4 antibody that blocks programmed cell death protein 1 and is approved against metastatic melanoma, squamous cell lung cancer, renal cell carcinoma, and Hodgkin's lymphoma after failure of prior line of chemotherapy. Here, we present a 40-year-old patient developing encephalopathy after treatment of Hodgkin's lymphoma with the immune checkpoint inhibitor nivolumab. In literature, cases of autoimmune encephalitis after receiving combination therapy of immune checkpoint inhibitors ipilimumab and nivolumab were described before. As far as we know, this is the unique case of encephalopathy reported after monotherapy with nivolumab treatment used for Hodgkin's lymphoma.

The efficacy of anti- IL-1 treatment in three patients with coexisting familial Mediterranean fever and multiple sclerosis.

It has been reported that multiple sclerosis (MS) is more common among patients with familial Mediterranean fever (FMF) than expected in normal population. Also, an association between MEFV gene variants and disease severity in MS has been described. Excessive production of interleukin-1 (IL-1) beta is responsible for FMF pathogenesis, and anti-IL-1 treatment is an effective approach in colchicine-resistant FMF patients. Here, we describe three patients with FMF and coexisting MS who have been treated with anti-IL-1 agents. Our observations suggest that blocking IL-1 is a safe and an effective alternative for colchicine resistant FMF and probably also for associated MS.

Restless Legs Syndrome in Patients with Behçet's Disease and Multiple Sclerosis: Prevalence, Associated Conditions and Clinical Features.

INTRODUCTION: To investigate the prevalence and characteristics of Restless Legs Syndrome (RLS) in patients with Behçet's Disease (BD) and Multiple Sclerosis (MS). METHODS: Consecutive patients with BD and MS seen in the outpatient clinic were included in the study. As a control group, volunteer subjects without a known peripheral or central nervous system disorder were included. The BD group was divided into two sub-groups as BD with neurological involvement [Neuro-Behçet's Disease (NBD)] and BD without any neurological involvement (other BD) for further evaluation. Data on demographic characteristics, medical history and family history were collected, and all patients underwent neurological examination. The patients were evaluated for the presence of diagnostic criteria for RLS. The features and severity of RLS were evaluated in patients with RLS using Restless Legs Syndrome Identification Form, and the International Restless Legs Syndrome Study Group (IRLSSG) Rating Scale. The clinical and radiological findings of patients with BD and MS were retrieved from their medical files. RESULTS: The study included a total of 96 patients with BD (mean age 39.9±11.8; 51 males; 41 patients with NBD) and 97 patients with MS (mean age 34.97±4.1 years; 24 males). There were 100 healthy control subjects (mean age 36.18±11.10 years; 46 males). RLS was more prevalent in MS (22.8%) and NBD (22%) groups compared to the control group (10%; p=0.004 and 0.029, respectively) with a statistically significant difference. The prevalence of RLS was higher in MS patients with less disability. Most severe RLS symptoms were observed in the MS group. The rate of sleep disorders was also higher in MS group. Although stress appeared to be a factor worsening RLS in all groups, its prevalence was higher in the MS group (p=0.011). There was no correlation between the distribution of magnetic resonance imaging lesions and RLS in both MS and NBD groups. CONCLUSIONS: It is well established that RLS can accompany disorders involving the peripheral and central nervous systems such as all types of peripheral neuropathy, myelopathy, and Parkinson's disease. The present study showed that MS and NBD also seem to be a risk factor for RLS, being associated with more severe symptoms.

Comparative analysis of fingolimod versus teriflunomide in relapsing-remitting multiple sclerosis.

BACKGROUND: Fingolimod and teriflunomide are commonly used in the treatment of relapsing-remitting multiple sclerosis (RRMS). These have not been compared in controlled trials, but only in observational studies, with inconclusive results. Comparison of their effect on relapse and disability in a real-world setting is therefore needed. OBJECTIVES: The objective of this study was to compare the efficacy of fingolimod and teriflunomide in reducing disease activity in RRMS. METHODS: This multicenter, retrospective observational study was carried out with prospectively collected data from 15 centers. All consecutive RRMS patients treated with teriflunomide or fingolimod were included. Data for relapses, Expanded Disability Status Scale (EDSS) scores and brain magnetic resonance imaging (MRI) scans were collected. Patients were matched using propensity scores. Annualized relapse rates (ARR), disability accumulation, percentage of patients with active MRI and treatment discontinuation over a median 2.5-year follow-up period were compared. RESULTS: Propensity score matching retained 349 out of 1388 patients in the fingolimod group and 349 out 678 in the teriflunomide group for final analyses. Mean ARR decreased markedly from baseline after 1 and 2 years of treatment in both the fingolimod (0.58-0.17 after 1 year and 0.11 after 2 years, p < 0.001) and teriflunomide (0.56-0.29 after 1 year and 0.31 after 2 years, p < 0.001) groups. Mean ARR was lower in fingolimod-treated patients than in those treated with teriflunomide at years 1 (p = 0.02) and 2 (p = 0.004). Compared to teriflunomide, the fingolimod group exhibited a higher percentage of relapse-free patients and a lower percentage of MRI-active patients after 2.5-year follow-up. Disability worsening was similar between the two groups. Patients were less likely to discontinue fingolimod than teriflunomide (p < 0.001). CONCLUSION: Fingolimod was associated with a better relapse control and lower discontinuation rate than teriflunomide. The two oral therapies exhibited similar effects on disability outcomes.

Intravenous Immunoglobulin Treatment for Recurrent Optic Neuritis.

INTRODUCTION: Recurrent optic neuritis neuritis (rON) is an autoimmune inflammatory condition of unknown cause. Intravenous immunoglobulin (IVIg) treatment is used for many autoimmune disorders; however we do not have any information about its effect in rON, other than case reports. We aimed to evaluate our patients with rON who were treated with IVIg. METHODS: Data from all our patients with rON with or without anti aquaporin4 (AQP4) seropositivity, seen between April 2011 and October 2015, who received IVIg treatment were retrospectively evaluated. RESULTS: Nine patients (all female) with rON had received IVIg. These patients were aged between 34 and 65 years, and had started receiving monthly IVIg from 6 to 58 months after onset of disease. In three out of nine rON patients serum AQP4 antibody were positive. Under current treatments the patients had continued to have attacks, therefore monthly IVIg was given in addition to the existing immunosuppressant drug. The follow up duration was between 6 to 31 months. Three patients, each suffered one relapse under IVIg treatment. Mean number of relapses in the year prior to treatment was 1.4±0.72, whereas it was 0.3±0.5 during the year after IVIg therapy. During follow-up with IVIg administration only one patient had fever and no other adverse events were reported. CONCLUSION: Monthly IVIg is well-tolerated and safe and it seems to be effective in rON as an add on treatment. However, since our study is a retrospective case series, future randomized controlled trials with IVIg are needed.