Typical trident sign and cardiac involvement in a patient suspected to Sarcoidosis despite negative whole-body FDG-PET: a case report.

BACKGROUND: Sarcoidosis is a systemic inflammatory disease histologically defined by the non-caseation granulomas formation in different organs, most commonly lungs, liver, skin, gastrointestinal system, eyes, neurologic and cardiac system CASE PRESENTATION: We report the case of a 42-year-old Gilaks woman who presented with myelopathy with characteristic MRI finding called trident sign. By finding this view in axial spinal Magnetic Resonance Imaging (MRI) imaging, a systemic evaluation was performed on the patient, which led to the diagnosis of cardiac involvement in Sarcoidosis with the specific appearance of this disease in cardiac MRI despite the negative Fluorodeoxyglucose (FDG)-positron emission tomography (PET) scan. CONCLUSIONS: Sometimes characteristic findings such as the trident sign prompt the physician to high suspicion and wide evaluation of the patient to reveal important organ involvement that changes the treatment decision and saves the patient.

Effect of vitamin A on recovery from the acute phase of multiple sclerosis-related optic neuritis, double-blind, randomized, placebo-controlled trial.

Background: Optic neuritis (ON) is one of the main neuro-ophthalmic presentations of multiple sclerosis (MS), and it causes optic nerve atrophy and axonal loss. However, so far, there is no effective treatment to improve long-term outcomes. Methods: In a double-blind placebo-controlled randomized clinical trial, 50 patients with MS-related ON were allocated into two arms (24 in the control group and 26 in the intervention group) receiving either 25000IU retinyl palmitate or an identical placebo for six months. Visual evoked potential (VEP), visual acuity, and the retinal nerve fiber layer (RNFL) thickness were evaluated and compared before and after the treatment. Results: RNFL thickness reduction in the affected eyes at sixth month compared to the baseline were 14.81 and 19.46 µm, in the intervention and control groups, respectively (P=0.017). However, VitA therapy did not affect visual acuity and VEP. Conclusion: Vitamin A supplementation in the patients with acute ON in MS could lessen optic nerve axonal loss.

Evaluation of liver injury in multiple sclerosis patients receiving pulsed steroid therapy.

OBJECTIVES: There is a paucity of literature about the methylprednisolone induced liver injury in multiple sclerosis (MS) patients. In this study, we intended to investigate the incidence, severity, and risk factors for liver injury in MS patients treated with pulsed methylprednisolone therapy. METHODS: This is a prospective observational study on MS patients treated with methylprednisolone pulses. All MS subjects with relapses who were referred to Sina Hospital between May 2020 to May 2021 were included in the study. They were evaluated for the demographic, clinical characteristics, and liver function tests. Liver injury was diagnosed if there was an elevation of serum aminotransferase levels above the upper normal limit (45 IU/L). RESULTS: A total of 314 individuals participated in the study. The prevalence of liver injury after treatment with pulsed methylprednisolone therapy was 2.86%. None of the cases with liver injury were severe. Univariate regression analysis demonstrated that the patients with liver injury had a significantly higher frequency of hyperlipidemia (p: 0.002), alcohol abuse (p: 0.021), and non-alcoholic fatty liver disease (NAFLD) (p: 0.005) compared to those without liver injury. Multivariate regression analysis showed that hyperlipidemia (p: 0.04, odds ratio (OR): 6.31), and history of alcohol abuse (p: 0.007, OR: 36.71) were significantly associated with liver injury. CONCLUSIONS: Our study highlights the importance of a close follow-up of the liver function tests in MS patients following pulsed methylprednisolone therapy, particularly in patients with NAFLD, hyperlipidemia, and history of alcohol-abusing.

Epidemiology of familial multiple sclerosis in Iran: a national registry-based study.

BACKGROUND: Admittedly, little is known about the epidemiological signatures of familial multiple sclerosis (FMS) in different geographical regions of Iran. OBJECTIVE: To determine the epidemiology and the risk of FMS incidence in several provinces of Iran with a different ethnic population including, Fars, Tehran, Isfahan (Persians), and Mazandaran (Mazanis), Kermanshah (Kurds), and Chaharmahal and Bakhtiari (Lors). METHODS: This cross-sectional registry-based study was performed on nationwide MS registry of Iran (NMSRI) data collected from 2018 to 2021. This system, registers baseline characteristics, clinical presentations and symptoms, diagnostic and treatments at regional and national levels. RESULTS: A total of 9200 patients including, 7003 (76.1%) female and 2197 (23.9%) male, were participated. About 19% of patients reported a family history of MS; the order from highest to lowest FMS prevalence was as follows: Fars (26.5%), Chaharmahal and Bakhtiari (21.1%), Tehran (20.5%), Isfahan (20.3%), Mazandaran (18.0%), and Kermanshah (12.5%). Of all FMS cases, 74.7% (1308 cases) were female and 25.3% (442 cases) were male. FMS occurrence was much more common in females than males (P-value = 0.001). Further, the mean age at onset was 30 years among FMS cases. A substantially higher probability of relapsing-remitting MS and secondary-progressive MS was found among FMS cases than sporadic MS (SMS) (P_value = 0.001). There was no significant difference in Expanded Disability Status Scale (EDSS) scores between FMS and SMS. The majority of FMS cases were observed among first-degree relatives, with the highest rate in siblings. There was a significant association between MS risk and positive familial history in both maternal and paternal aunt/uncle (P_value = 0.043 and P_value = 0.019, respectively). Multiple sclerosis occurrence among offspring of females was higher than males (P_value = 0.027). CONCLUSIONS: In summary, our findings imply a noteworthy upward trend of FMS in Iran, even more than the global prevalence, which suggests a unique Atlas of FMS prevalence in this multi-ethnic population. Despite the highest rate of FMS within Persian and Lor ethnicities, no statistically significant difference was observed among the provinces.

Disease-modifying therapies and T1 hypointense lesions in patients with multiple sclerosis: A systematic review and meta-analysis.

BACKGROUND: Previous research has shown that cerebral T1 hypointense lesions are positively correlated with the disability of multiple sclerosis (MS) patients. Hence, they could be used as an objective marker for evaluating the progression of the disease. Up to this date, there has not been a systematic evaluation of the effects of disease-modifying therapies (DMTs) on this prognostic marker. OBJECTIVES: To evaluate the effects of FDA-approved DMTs on the numbers and volume of T1 hypointense lesions in adult patients with MS. METHODS: We included studies with the mentioned desired outcomes. In March 2021, we searched MEDLINE (Ovid), Embase, and CENTRAL to find relevant studies. All included studies were assessed for the risk of bias using the RoB-2 tool. Extracted data were analyzed using a random-effects model. Certainty of evidence was assessed using GRADE. RESULTS: Thirteen studies with 7484 participants were included. Meta-analysis revealed the mean difference between the intervention and comparator groups for the number of lesions was -1.3 (95% CI: -2.1, -0.5) and for the mean volume of lesions was -363.1 (95% CI: -611.6, -114.6). Certainty of evidence was judged to be moderate. Heterogeneity was considerable. DISCUSSION: DMTs reduce the number and volume of T1 hypointense lesions. Although, these findings must be interpreted cautiously due to the high values of heterogeneity.

Safety and efficacy of fingolimod in Iranian patients with relapsing-remitting multiple sclerosis: An open-label study.

BACKGROUND: Fingolimod was the first oral therapy approved for treating relapsing-remitting multiple sclerosis (RRMS) in 2010. This open-label study evaluated the safety and efficacy of fingolideR, 0.5 mg in Iranian MS patients during one-year follow-up. METHODS: A multicenter, open-label, longitudinal was designed to evaluate the safety and efficacy of fingolideR, 0.5 mg over a one-year follow-up period across 11 centers. The patients were visited by their neurologists every two months to evaluate possible adverse events and clinical disease activity considered by recording Kurtzke's Expanded Disability Status Scale (EDSS). RESULTS: A total of 252 patients with the mean treatment duration of 343±45.70 days were. 20 patients experienced adverse events (AEs) and serious adverse events (SAEs) such as resistant urinary tract infection (UTI), premature atrial contraction (PAC), skin allergic reaction, macular edema, chicken pox, zona, panic attacks, and exacerbations associated with steroids treatment, all of which led to FingolideR discontinuation. The mean EDSS decreased from (2.15±1.29, 95%CI: 1.99to2.32) at baseline to (1.85±1.22, 95%CI: 1.68to2.02) at 12th month (final visit) while a p-value revealed significant differences comparing baseline and final EDSS (p<0.001). Mean annualized relapse rate (ARR) of the patients in one year prior to the study was (0.006±0.016, 95%CI: 0.004to0.008) which changed to (0.005±0.016, 95%CI: 0.003to0.007) at the end of the study period. Patients with a 12-month period of fingolideR treatment experienced sustained ARR and disease progression (p<0.001). CONCLUSION: The obtained findings suggest that the administration of FingolideR, 0.5 mg (Fingolimod, Osvahpharma, Tehran, Iran) is safe and efficient for Iranian MS patients.

B-cell depleting therapies may affect susceptibility to acute respiratory illness among patients with multiple sclerosis during the early COVID-19 epidemic in Iran.

OBJECTIVE: To determine whether the course of COVID-19 is more severe in patients with MS and if MS disease-modifying treatments (DMTs) affect the risk of contracting the disease. METHODS: In a cross-sectional survey, data were collected by sending a questionnaire to 2000 patients with a demyelinating disease through an online portal system. Collected data included the current MS DMT and patient-reported disability level, history of recent sick contact, recent fever, respiratory symptoms, diagnosis with COVID-19, and the disposition after the diagnosis. We defined a COVID-19-suspect group as patients having fever and cough or fever and shortness of breath, or a presumptive diagnosis based on suggestive chest computed tomography. We calculated the proportion of COVID-19-suspect patients and compared their demographics, clinical characteristics, and DMT categories with the rest of survey-responders, using univariable and multivariable models. RESULTS: Out of 712 patients, 34 (4.8%) fulfilled our criteria for being in the COVID-19-suspect group. Only two patients required hospitalization. No patient required intensive care. In a multivariable model, disease duration (p-value=0.017), DMT category (p-value=0.030), and history of sick contact (p-values<0.001) were associated with the risk of being in the COVID-19-suspect group. Being on B-cell depleting antibodies (as compared to non-cell depleting, non-cell trafficking inhibitor DMTs) was associated with a 2.6-fold increase in the risk of being in the COVID-19-suspect group. (RR: 3.55, 95%CI: 1.45, 8.68, p-value=0.005). CONCLUSIONS: The course of infection in patients with MS suspected of having COVID-19 was mild to moderate, and all patients had a full recovery. B-cell depleting antibodies may increase the susceptibility to contracting COVID-19.

Persian Adaptation of Actionable Bladder Symptom Screening Tool Among Patients with Multiple Sclerosis.

PURPOSE: Multiple sclerosis (MS) is the most neurologic disease among individuals of 20-45 years. About 75% of MS patients report bladder problems that have a moderate-high impact on their life. The present study aimed to translate and determine the validity and reliability of the Persian version of ABSST questionnaire. METHODS: The standard validation process for preparing the Persian version of ABSST was performed by means of an expert committee. After a pilot study and confirming the harmonized translated form, we tested the final version of questionnaire on 40 patients with a definite diagnosis of MS symptoms, once at the baseline and another after two weeks in order to prevent recall-induced agreement. Test-retest reliability was calculated as Spearman's correlation coefficient. Also, content validity indices (CVIs), as well as internal consistency were analyzed in STATA. RESULTS: Forty participants with a mean age of 38.8 years were included in this study. Only 20% of them were male. The Persian version achieved a good internal consistency with a Cronbach-α value of 0.91, relatively similar to the original version. The coefficients for measuring the correlation between each item score with the total score of our questionnaire were between 0.58-0.89. This value confirmed an appropriate validity for the Persian version of ABSST. Regarding test-retest reliability assessment, total Spearman`s coefficient was 0.85; with 0.84 for the severity of symptoms and 0.87 for the impact of urologic disorders on patients` social life. CONCLUSION: The current findings proved that this accurate questionnaire could be used to investigate urinary symptoms among Persian-speaking MS patients.

Association of cigarette smoking with neuromyelitis optica-immunoglobulin G sero-positivity in neuromyelitis optica spectrum disorder.

Background: Neuromyelitis optica spectrum disorder (NMOSD) is a neuroinflammatory demyelinating disease caused by the presence of a highly specific serum autoantibody marker, NMO-immunoglobulin G (NMO-IgG), that reacts against the water channel aquaporin-4 (AQP4). The present study examined the association between NMO-IgG sero-positivity and environmental factors such as cigarette smoking. Methods: A cross-sectional study was conducted in Sina Hospital, a tertiary referral center in Tehran, Iran. All the patients with a definite diagnosis of NMOSD were involved in this study. The enzyme-linked immunosorbent assay (ELISA) was used to examine the AQP4-IgG status. To assess the association between NMO-IgG sero-positivity and cigarette smoking, a researcher-made questionnaire covering patients' lifestyle information on smoking habits was designed and administered using the structured face-to-face interviews with the patients. Results: The positive and negative NMO-IgG results were found in 44 (46.8%) and 50 (53.2%) patients, respectively. The increased NMO-IgG sero-positivity odds were observed among the lifetime smokers [odds ratio (OR) = 3.24, 95% confidence interval (CI): 1.16-9.08], current smokers (OR = 6.08, 95% CI: 1.26-29.39), and passive smokers (OR = 2.22, 95% CI: 1.10-4.50). Conclusion: Lifetime and current smoking as well as passive smoking can be regarded as risk factors for NMO-IgG sero-positivity. Smoking with its immunological effects can lead to the production of autoantibodies such as NMO-IgG.

The association between dietary sugar intake and neuromyelitis optica spectrum disorder: A case-control study.

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon autoimmune disease of the central nerves system (CNS) by inflammatory nature. The effects of high dietary sugar intake on inflammation and dysbiosis have been received more attention in recent years. The aim of the present study was to investigate the association between various types of dietary sugar intake and NMOSD odds and clinical features. METHOD: The current case-control study was conducted among 70 patients with definite NMOSD diagnosis based on 2015 international consensus criteria and 164 hospital-based controls. Demographic and anthropometric information in all participants and disease characteristics just in case group were obtained. Dietary data during the past year of study attendance was collected by a validated 168-item food frequency questionnaire. Participants were stratified into 3 tertiles according to each type of sugar intake and the third tertile considered as reference in multivariate regression models. The correlation between dietary sugar and disease features were analyzed using Pearson correlation test. RESULTS: The mean ± SD of total sugar intake increased from 80.73 ± 17.71 to 208.71 ± 57.93 g/day across tertiles of total sugar intake. In fully adjusted model, lower intake of sugar was associates with decreased odds of NMOSD in the first tertile vs third tertile by ORs of: 0.02(CI:0.00-0.08; p-for-trend:0.00), 0.02(CI:0.00-0.10; p-for-trend:0.00), 0.23(CI:0.08-0.61; p-for-trend:0.00), 0.19(CI:0.06-0.58; p-for-trend:0.00) and 0.16(CI:0.05-0.51; p-for-trend:0.00) for glucose, fructose, galactose, lactose and sucrose, respectively. The odds of NMOSD had a 1.72-fold (CI: 1.43-2.03; p-for-trend:0.00) significant raise per every 10 g increase for total sugar intake. There was no significant correlation between various types of dietary sugar intakes and relapse rate or patients' disability. CONCLUSION: The present study proposes a possible direct association between high intake of various sugar types and odds of suffering from NMOSD. More investigations are needed to prove this results.

The effect of traditional dysphagia therapy on the swallowing function in patients with Multiple Sclerosis: A pilot double-blinded randomized controlled trial.

BACKGROUND: Dysphagia is common following Multiple Sclerosis (MS) and is associated with significant morbidity and mortality. The current rehabilitation program to swallowing therapy is Traditional Dysphagia Therapy (TDT), but there is a dearth of evidence about its effectiveness in MS patients. OBJECTIVES: This study was aimed to determine the effects of the TDT on the swallowing function in MS patients with dysphagia. METHODS: A pilot double blind randomized clinical trial was carried out on 20 patients with MS. Patients were randomly divided into experimental group (TDT) comprising sensorimotor exercises and swallowing maneuvers, and Usual Care (UC) comprising diet prescription and postural changes. Patients in both groups received treatments for 6 weeks, 18 treatment sessions, 3 times per week, every other day. The Mann Assessment of Swallowing Ability (MASA) was the main outcome measure. The swallowing ability was assessed before treatment (T0), after the end of 9th session (T1), after the end of 18th session (T2), and after 6 weeks follow-up (T3). Penetration-Aspiration Scale (PAS) and Pharyngeal Residue Rating Scale (PRRS) as secondary outcome measures were applied at T0 and T2. RESULTS: Both groups had improved regarding MASA, PAS and PRRS scores over the time (P < 0.001). The improvements achieved in all outcomes were significantly greater in the TDT group than those of the UC group. The Main effect of the Time × Group interaction was significant for MASA score (P < 0.001). The large effect sizes were found for MASA score in both the TDT (d = 3.91) and the UC (d = 1.11) groups. CONCLUSIONS: This pilot randomized controlled trial showed that the TDT significantly improved the swallowing function of the MS patients with dysphagia.

Autoimmune diseases associated with Neuromyelitis Optica Spectrum Disorders: A literature review.

INTRODUCTION: Neuromyelitis Optica (NMO) is an autoimmune inflammatory demyelinating disease of the central nervous system (CNS) which predominantly involves optic nerves and spinal cord. Since the introduction of Neuromyelitis Optica Spectrum Disorders (NMOSD) as a separate entity, there have been many reports on its association with other disorders including systemic and organ-specific autoimmune diseases. Here, we reviewed other immune-mediated diseases associated with NMOSD and tried to categorize them. METHODS: The present review was conducted using the PUBMED database based on papers from 1976 (i.e., since the first NMO comorbidity with SLE was reported) to 2017. We included all articles published in English. The keywords utilized included Neuromyelitis optica, Neuromyelitis Optica Spectrum Disorders, Devic's disease, in combination with comorbidity or comorbidities. RESULTS: Diseases with immune-based pathogenesis are the most frequently reported co-morbidities associated with NMOSD, most of which are antibody-mediated diseases. According to literature, Sjogren's Syndrome (SS) and Systemic Lupus Erythematosus (SLE) are the most frequently reported diseases associated with NMOSD among systemic autoimmune diseases. Further, myasthenia gravis in neurological and autoimmune thyroid diseases in non-neurological organ-specific autoimmune diseases are the most reported comorbidities associated with NMOSD in the literature. CONCLUSIONS: NMOSD may be associated with a variety of different types of autoimmune diseases. Therefore, systemic or laboratory signs which are not typical for NMOSD should be properly investigated to exclude other associated comorbidities. These comorbidities may affect the treatment strategy and may improve the patients' care and management.

Evaluation of the risk of cervical cancer in patients with Multiple Sclerosis treated with cytotoxic agents: A cohort study.

Background: Since most patients with relapsing-remitting multiple sclerosis (RRMS) are women, the present study aimed to determine whether treatment of patients with MS by cytotoxic agents is associated with an increased risk of cervical dysplasia. Cancer screening is often neglected in the chronic diseases such as MS, so more attention in this field was needed. Decreasing morbidity and mortality due to cervical cancer is the most important goal of screening in female MS patients especially in child bearing age. Thus, it can be said that this is the first study which investigated this important issue. Methods: A total of 129 individuals participated in this cohort study. They were assigned into 3 groups including 43 patients with MS who were treated with cytotoxic drugs, 43 patients with MS on immunomodulators, and 43 normal healthy controls. Pap smears were performed following standard methods and the results obtained from the three groups were compared by statistical analysis. Demographic data, Expanded Disability Status Scale (EDSS), and Pap smear changes were analyzed by SPSS software. Results: The most commonly detected abnormality in all examined patients and healthy controls was inflammation. Five patients with MS who were treated with cytotoxic agents revealed benign cellular changes (BCC) in their Pap smear that were statistically significant in comparison with other groups (P = 0.03). Patients who took Mitoxantrone presented BCC more than other groups [Odds ratio (OR) = 9.44, 95% confidence interval (CI): 1.46-60.70]. There was no significant difference between mean duration of MS diagnosis (P = 0.12), mean duration of previous MS treatments (P = 0.25), and mean duration of current MS treatments (P = 0.21) in patients with BCC compared to normal healthy controls or inflammatory change. Conclusion: According to the results of present study, BCC is more frequently observed in patients with MS who were treated with cytotoxic agents with immunosuppressive effect. Since BCC is a 'premalignant condition', the authors suggest that mandatory annual Pap smear should be performed for patients with MS who are treated with cytotoxic agents irrespective of their age in order to detect early signs of malignancy.

Anti-JC virus antibody sera positivity and index value among patients with multiple sclerosis may be correlated with age, sex, and area of residence.

Anti-JC virus (JCV) antibody index is the predictive factor of progressive multifocal leukoencephalopathy (PML) for multiple sclerosis (MS) patients treating with natalizumab. The aim of this study is to evaluate the prevalence of anti-JCV antibody positivity and index among Iranian patients who are the candidate for natalizumab and its correlation with their demographic data and previous therapies. A cross-sectional design was assessed for receiving anti-JCV antibody test results between January 2014 and December 2016. Demographic data and disease characteristics were also obtained. Statistical analysis and logistic regression were done using SPSS. Among 803 MS patients that were observed, the prevalence of anti-JCV antibody positivity was 67.9% (mean of index = 2.23 ± 1.16) and 67.6% of positive patients had an index ≥ 1.5. Males were more antibody positive than females (81.7 and 64% respectively; significance (sig.) < 0.001, OR = 2.51, CI 1.65-3.81). The rate of positivity was lower in patients under the age of 18. Patients who lived in cold regions had significantly more prevalence of positivity (Num. = 403; sig. = 0.043 and OR = 1.86; CI 1.02-3.39) and with higher rate of index ≥ 1.5 (sig. = 0.017; OR = 3.99, CI 1.79-8.88). Disease onset age between 28 and 37 years were more positive compared to 18-27 years (N = 480; sig. = 0.02; OR = 1.85, CI 1.09-3.14). Age, male gender, onset age, and cold area of residency significantly influenced anti-JCV antibody sera positivity. Only age of onset and cold area of residency were related to the index. No significant difference was observed between type, dosage, and duration of previous immunosuppressant drugs and anti-JCV antibody positivity and index value.

Environmental risk factors in neuromyelitis optica spectrum disorder: a case-control study.

Neuromyelitis optica spectrum disorder (NMOSD) has unknown risk factors. The aim of this study was to identify the environmental risk factors for NMOSD. A case-control study was conducted in Tehran from 2015 to 2016 among 100 patients with NMOSD. Sex-matched healthy controls (n = 400) were selected through random digit dialing (RDD). Logistic regression was used to estimate unadjusted and adjusted ORs (odds ratio) at 95% confidence intervals (CI) via SPSS. Compared with the control population, in NMOSD patients, the adjusted OR for low dairy consumption per week was (OR = 18.09; 95% CI 6.91, 47.37), following low sea food intake (OR = 13.91; 95% CI 6.13, 31.57) and low fruit and vegetable consumption (OR = 6.23; 95% CI 3.07, 12.62). The lower heavy physical activity (OR: 16.11, 95% CI 7.03, 36.91) among patients had risen the risk of NMOSD. A past history of head trauma was considered a risk for NMOSD (OR: 8.39, 95% CI 4.97, 14.16). The association between NMOSD and intentional abortion only among females (OR: 7.42, 95% CI 2.81, 19.55) was detected. This study indicates significant associations between dietary habits, life style, history of head trauma and intentional abortion in female and the later diagnosis of NMOSD.

Diagnosis and management of Neuromyelitis Optica Spectrum Disorder (NMOSD) in Iran: A consensus guideline and recommendations.

Neuromyelitis Optica Spectrum Disorder (NMOSD) is a relapsing neuro inflammatory disease of the central nervous system that typically presents with optic neuritis or myelitis and may cause severe disability. The diagnostic criteria have been updated and several immunosuppressive agents have been demonstrated to prevent acute exacerbations. As the disease rarely develops in a progressive course, management of acute attacks and proper prevention of exacerbations may change the long term out-come and prevent future disability. Consensus recommendations and guidelines will help the physicians to improve their practice and unify the treatment approaches in different communities. In order to develop a national consensus and recommendations for the diagnosis and management of NMOSD in Iran, a group of neurologists with long term experience in management of NMOSD were gathered to develop this consensus based on available national and international data. The primary draft was prepared and discussed to suggest the most appropriate treatment for these patients. We propose strategies for early diagnosis and treatment for prevention of relapses and minimizing consequences of attacks as a primary therapeutic goal. Attacks are currently treated with intravenous corticosteroids and, in refractory cases, with plasma exchange. All participants agreed on preventive treatment with currently available immunosuppressive agents such as azothioprin, rituximab and mycofenolate mofetil based on previous positive data in NMOSD in order to reduce attack frequency. The current consensus reviews the previous data and provides the clinicians with practical recommendations and advices for the diagnosis and management of NMOSD based on scientific data and clinical experience.

Neuromyelitis optica spectrum disorders in Iran.

BACKGROUND: Neuromyelitys optica spectrum disorder (NMOSD) is a rare demyelinating disease; as a result, the epidemiological data on this disorder is scarce. In this regard, the aim of this study was to estimate the prevalence, serology, and clinical features of NMOSD in Caucasian population in Tehran, Iran. METHOD: A cross sectional study was performed in Tehran from 2015 to 2016 among patients registered with NMOSD diagnosis, based on consensus criteria published in 2015. The researchers designed a questionnaire to cover the important epidemiological and clinical data of NMOSD in Tehran. Structured face to face interviews were conducted with 103 patients by trained interviewers to collect the data. The logistic regression was applied in analysis via SPSS software package. RESULT: The prevalence of NMOSD in Tehran was 0.86 per 100,000 in 2016. Female to male ratio was 5:1 with mean age at the disease onset of 31.54. NMO-IgG were positive in 44 (46.8%) patients, and the primary presenting symptoms of TM were observed in 29 (28.2%) patients. The adjusted odds ratio for sex was estimated for depression (OR = 6.83; 95% CI: 1.47, 31.71), migraine (OR = 1.27; 95% CI: 1.13, 1.42), and hypothyroidism (OR = 1.25; 95% CI: 1.12, 1.39). CONCLUSION: The researchers indicated that the rate of NMOSD is significantly higher among females and younger age group. In addition, the history of depression, migraine, and hypothyroidism has been observed more among female patients in comparison to male patients.

Cross-cultural adaption and validation of the Persian version of the SWAL-QOL.

The aim of this study was to translate and cross-culturally adapt the swallowing quality-of-life questionnaire (SWAL-QOL) to Persian language and to determine validity and reliability of the Persian version of the swallow quality-of-life questionnaire (PSWAL-QOL) in the patients with oropharyngeal dysphagia.The cross-sectional survey was designed to translate and cross-culturally adapt SWAL-QOL to Persian language following steps recommended in guideline. A total of 142 patients with dysphagia (mean age = 56.7 ±â€Š12.22 years) were selected by non-probability consecutive sampling method to evaluate construct validity and internal consistency. Thirty patients with dysphagia were completed the PSWAL-QOL 2 weeks later for test-retest reliability.The PSWAL-QOL was favorably accepted with no missing items. The floor effect was ranged 0% to 21% and ceiling effect was ranged 0% to 16%. The construct validity was established via exploratory factor analysis. Internal consistency was confirmed with Cronbach α >0.7 for all scales except eating duration (α = 0.68). The test-retest reliability was excellent with intraclass correlation coefficient (ICC) ≥0.75 for all scales.The SWAL-QOL was cross-culturally adapted to Persian and demonstrated to be a valid and reliable self-report questionnaire to measure the impact of dysphagia on the quality-of-life in the Persian patients with oropharyngeal dysphagia.

The expression of VDR mRNA but not NF-κB surprisingly decreased after vitamin D treatment in multiple sclerosis patients.

BACKGROUND AND PURPOSE: The aim of this study was to investigate the expression levels of vitamin D receptor (VDR) and NF-κB mRNAs in vitamin D (VD) supplemented multiple sclerosis (MS) patients. METHODS: RRMS patients received 50,000 IU vitamin D3/week as an intra-muscular injection for 2 months. Blood samples were obtained from 30 MS patients before and after VD supplementation and 32 healthy individuals, and then VDR and NF-κB mRNA levels were measured by real time PCR method and analyzed with independent and paired t-tests. Moreover, some correlations were performed between the expression levels of selected genes and some clinical features of MS and control groups. RESULTS: Surprisingly, the expression level of VDR mRNA significantly decreased after 2 months supplementation with VD in our selected patients and in contrast, the level of serum 25(OH) D increased after supplementation. Although, we didn't find any significant difference in the expression level of NF-κB gene before and after treatment with VD, its expression significantly decreased in untreated MS cases compared with healthy controls. CONCLUSION: In conclusion, we found some new evidences from the molecular mechanism of vitamin D effectiveness in MS treatment. Also, we need more functional studies to find the effect of VD on the expression level of VDR mRNA.