Bagel sign in neuro Behçet's disease myelitis: neuroimages.

Neuro-Behçet's disease (NBD) is a chronic heterogenous autoimmune disorder. It may involve central or pripheral nervous system but rarely shows spinal cord involvement (SCI). Bagel Sign is a unique sign of SCI due to NBD. It is a central T2W hyperintense lesion with a hypointense core on axial magnetic resonance imaging (MRI). This sign may be a complete or an incomplete ring with or without post-contrast enhancement. Here we report a patient with NBD whose primary presentaion was transverse myelitis. A 14-year-old patient was admitted due to triparesis and urinary retention. He had T2W hyperintensities from the left basal ganglion down to the T10 level. A similar anteromedial spot has been described in anterior spinal cord infarction which favors an ischemic pathogenesis for NBD. To our knowledge this is the first report of Bagel Sign and longitudinally extensive transverse myelitis with gray matter involvement.

Metal concentrations in cerebrospinal fluid, blood, serum, plasma, hair, and nails in amyotrophic lateral sclerosis: A systematic review and meta-analysis.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with progressive muscle wasting, paralysis, and respiratory failure. Whereas approximately 10-15 % of ALS cases are familial, the etiology of the remaining, sporadic ALS cases remains largely unknown. Environmental exposures have been suggested as causative factors for decades, and previous studies have found elevated concentrations of metals in ALS patients. PURPOSE: This meta-analysis aims to assess metal concentrations in body fluids and tissues of ALS patients. METHODS: We searched the MEDLINE and EMBASE databases on December 7th, 2022 for cross-sectional, case-control, and cohort studies which measure metal concentrations in whole blood, blood plasma, blood serum, cerebrospinal fluid (CSF), urine, erythrocytes, nail, and hair samples of ALS patients. Meta-analysis was then performed when three or more articles existed for a comparison. FINDINGS: Twenty-nine studies measuring 23 metals were included and 13 meta-analyses were performed from 4234 screened entries. The meta-analysis results showed elevated concentrations of lead and selenium. Lead, measured in whole blood in 6 studies, was significantly elevated by 2.88 µg/L (95 % CI: 0.83-4.93, p = 0.006) and lead, measured in CSF in 4 studies, was significantly elevated by 0.21 µg/L (95 % CI: 0.01 - 0.41, p = 0.04) in ALS patients when compared to controls. Selenium, measured in serum/plasma in 4 studies, was significantly elevated by 4.26 µg/L (95% CI: 0.73 - 7.79, p = 0.02) when compared to controls.Analyses of other metal concentrations showed no statistically significant difference between the groups. CONCLUSION: Lead has been discussed as a possible causative agent in ALS since 1850. Lead has been found in the spinal cord of ALS patients, and occupational exposure to lead is more common in ALS patients than in controls. Selenium in the form of neurotoxic selenite has been shown to geochemically correlate to ALS occurrence in Italy. Although no causal relationship can be established from the results of this meta-analysis, the findings suggest an involvement of lead and selenium in the pathophysiology of ALS. After a thorough meta-analysis of published studies on metal concentrations in ALS it can only be concluded that lead and selenium are elevated in ALS.

A Randomized Controlled Trial of the Effect of Repetitive Transcranial Magnetic Stimulation of the Motor Cortex on Lower Extremity Spasticity in Hereditary Spastic Paraplegia.

INTRODUCTION: Hereditary spastic paraplegia refers to a group of conditions characterized by a slow progression of spasticity in lower limbs resulting in gait abnormalities. Current treatment options have proven to be insufficient in terms of symptom alleviation. In this study, we tested the effectiveness of repetitive transcranial magnetic stimulation (rTMS) on lower limb spasticity in patients with hereditary spastic paraplegia. METHODS: Eight patients were randomly assigned to receive either five sessions of active 5 Hz-rTMS ( n = 4) or sham rTMS ( n = 4). The primary outcome was a change in spasticity assessed by the modified Ashworth scale. Secondary outcomes were change in 10 m walking test, Fugl-Meyer assessment of lower extremity motor function, and quality-of-life short-form survey scores. Assessment of the outcomes was done before, upon completion, and 1 month after the intervention. We analyzed the data using repeated-measure analysis of variance. RESULTS: Mean age of the participants was 38.5 (SD = 5.4) years, and 50% were women. Compared with sham rTMS, real rTMS was effective in decreasing modified Ashworth scale (rTMS × time: F [df = 2] = 7.44; P = 0.008). Real rTMS group had lower modified Ashworth scale scores at the end of rTMS sessions (estimate = -0.938; SE = 0.295; P = 0.019) and at the end of follow-up (estimate = -0.688; SE = 0.277; P = 0.048) compared with the sham rTMS group. Real and sham rTMS groups were not different in the secondary outcomes. CONCLUSIONS: Repetitive transcranial magnetic stimulation is an effective method in reducing lower limb spasticity of patients with hereditary spastic paraplegia.

Adverse drug reactions of Rituximab in patients suffering from autoimmune neurological diseases.

BACKGROUND: Rituximab, a chimeric human/mouse monoclonal antibody targeting CD-20 antigens, has been used recently for various rheumatological and autoimmune diseases, including autoimmune neurological disorders. OBJECTIVES: We aimed to study the frequency, seriousness, causality, and preventability of adverse drug reactions (ADRs) of rituximab in Iranian patients with autoimmune neurological diseases. METHODS: In this cross-sectional observational study, patients with autoimmune neurological diseases who had an indication for rituximab treatment were enrolled. Naranjo adverse drug reaction probability scale was used to assess the causality of ADRs, and the preventability of the ADRs was determined by P-Method. The seriousness of ADRs was also determined. RESULTS: A total of 264 ADRs were recorded from 97 patients. The Median (min-max) number of ADRs experienced by patients was 3 (1-7) events. 11.3% of patients experienced serious ADRs. 18.2% and 26.9% of ADRs were Definite and Probable, respectively. Only 5% of the ADRs were ''preventable". The most frequent ADRs were rituximab infusion-related reactions. CONCLUSION: Rituximab had an acceptable safety profile in our study patients. However, there must be certain cautions regarding the use of the medication for the elderly or patients with a compromised immune system. Timely detection and management of ADRs would also be crucial to prevent severe and permanent damages. Moreover, considering that rituximab is used as an off-label treatment for autoimmune neurological diseases, a risk-benefit assessment would be necessary before deciding on the treatment choice.

Clinical features and outcomes of patients with myasthenia gravis affected by COVID-19: A single-center study.

INTRODUCTION: Myasthenia Gravis (MG) is an autoimmune disorder that can exacerbate for various reasons including infections. In this study, we describe clinical symptoms, outcomes, and management of MG patients affected by COVID-19 infection. METHODS: This observational retrospective study was performed on patients previously diagnosed as MG, presenting with COVID-19 in the clinic or emergency department between April 2020 and August 2021. The clinical data, outcome, and therapeutic interventions were assessed in 83 patients with MG and COVID-19 infection. RESULTS: Seventy-seven patients performed PCR testing for COVID-19, of which 73 (94.8 %) were positive. Seven patients had the positive serologic test for COVID-19 (IgG and IgM). Fifty-seven (68.7 %) patients had lung involvement. Thirty-five (42.1 %) of patients were admitted to the hospital. Twelve (14.5 %) patients needed hospitalization in an intensive care unit (ICU), with a mean stay of 7.36 ± 5.6 days (rang: 2-20 days). Four (4.8 %) patients were intubated and required mechanical ventilation. Sixteen (19.3 %) patients experienced an exacerbation of myasthenia gravis and were treated with PLEX (n = 2), IVIG (n = 7), and intravenous (IV) methylprednisolone (n = 7). The outcome was favorable in 79 patients and fatal in four patients, three of whom had other comorbidities. One patient died due to severe COVID-19 involvement. CONCLUSION: The findings from our study demonstrated that patients with previous MG concurrence with COVID-19 have favorable clinical outcomes. Most patients did not need to be hospitalized and more than 80 % of patients did not display MG exacerbation.

Preventive effect of greater occipital nerve block on patients with episodic migraine: A randomized double-blind placebo-controlled clinical trial.

OBJECTIVE: Since the data regarding the efficacy of greater occipital in episodic migraines are rare, we aimed to examine the efficacy of greater occipital block in the prophylaxis of episodic migraines without aura and compare different injectable drug regimens. METHODS: In a randomized, double-blind placebo-controlled trial, adult patients suffering from episodic migraines without aura were randomized to one of the following: triamcinolone, lidocaine, triamcinolone plus lidocaine, and saline. Patients were assessed at baseline, one week, two weeks, and four weeks after the injection for severity and duration of headaches and side effects. RESULTS: Fifty-five patients completed the study. Repeated measures ANOVA indicated that the severity and duration decreased significantly after the greater occipital block (P < 0.001, P = 0.001 respectively) in all four groups. However, there was no difference between groups at any study time points (P > 0.05). In paired sample T-test, only groups 2 and 3 with lidocaine as a part of the injection showed a significant decrease in frequency compared to the baseline (P = 0.002, P = 0.019). Three patients reported side effects with a possible association with triamcinolone. CONCLUSION: Greater occipital block with a local anesthetic significantly decreases the number of attacks in episodic migraine, whereas no injection was superior to the placebo in regards to the duration and severity of the headaches.Trial Registration Information: Iranian Registry of Clinical Trials (IRCT). Registration number: IRCT2017070334879N1. https://www.irct.ir/trial/26537.

Molecularly imprinted polypyrrole@CuO nanocomposite as an in-tube solid-phase microextraction coating for selective extraction of carbamazepine from biological samples.

A nanocomposite of molecularly imprinted polypyrrole on copper oxide (MIP@CuO) was introduced as a new coating for in-tube solid-phase microextraction (IT-SPME). The method coupled with HPLC-UV was successfully applied for analysis of carbamazepine (anticonvulsant and bipolar disorder medication) in biological samples. First, in order to increase the surface area and stability of the coating, copper oxide (CuO) nanosheets were synthesized on the inner surface of a copper tube using a chemical method. Then, molecularly imprinted polypyrrole coating (using carbamazepine as a template) was deposited on CuO by a facile in-situ electrodeposition method. According to the results, The MIP@CuO coating shows long life time, enhanced extraction efficiency, and good clean-up, for pre-concentration and determination of carbamazepine in biological samples. The synthesized adsorbent also showed high selectivity to carbamazepine compared to other drugs with similar structure. Important factors affecting the extraction efficiency of the analyte in the in-tube SPME method, such as salt concentration, extraction and desorption times, flowrates of the sample solution, and eluent, were optimized. Under optimal conditions, the method showed good linearity for carbamazepine in the range of 0.05-500 µg L-1, 0.10-500 µg L-1, and 0.10-500 µg L-1 in water, urine, and plasma samples, respectively, with coefficients of determination better than 0.996. The limits of detection were in the range of 0.01-0.05 µg L-1 in different matrices. The intra- and inter-assay precisions (RSD%, n = 3) were in the range of 6.7-8.1 % and 7.1-9.5 %, respectively.

Guillain-Barre Syndrome and COVID-19 Vaccine: A Report of Nine Patients.

INTRODUCTION: Guillain-Barre Syndrome (GBS) is an autoimmune acute inflammatory demyelinating polyneuropathy usually elicited by an upper respiratory tract infection. Several studies reported GBS associated with Coronavirus Disease 2019 (COVID-19) infection. In this study, we described nine GBS patients following the COVID-19 vaccine. METHODS: In this study, nine patients were introduced from six referral centers for neuromuscular disorders in Iran between April 8 and June 20, 2021. Four patients received the Sputnik V, three patients received the Sinopharm, and two cases received the AstraZeneca vaccine. All patients were diagnosed with GBS evidenced by nerve conduction studies and/or cerebrospinal fluid analysis. RESULTS: The median age of the patients was 54.22 years (ranged 26-87 years), and seven patients were male. The patients were treated with Intravenous Immunoglobulin (IVIg) or Plasma Exchange (PLEX). All patients were discharged with some improvements. CONCLUSION: The link between the COVID-19 vaccine and GBS is not well understood. Given the prevalence of GBS over the population, this association may be coincidental; therefore, more studies are needed to investigate a causal relationship.

BVVL/ FL: features caused by SLC52A3 mutations; WDFY4 and TNFSF13B may be novel causative genes.

Brown-Vialetto-Van Laere (BVVL) and Fazio-Londe are disorders with amyotrophic lateral sclerosis-like features, usually with recessive inheritance. We aimed to identify causative mutations in 10 probands. Neurological examinations, genetic analysis, audiometry, magnetic resonance imaging, biochemical and immunological testings, and/or muscle histopathology were performed. Mutations in known causative gene SLC52A3 were found in 7 probands. More importantly, only 1 mutated allele was observed in several patients, and variable expressivity and incomplete penetrance were clearly noted. Environmental insults may contribute to variable presentations. Putative causative mutations in other genes were identified in 3 probands. Two of the genes, WDFY4 and TNFSF13B, have immune-related functions. Inflammatory responses were implicated in the patient with the WDFY4 mutation. Malfunction of the immune system and mitochondrial anomalies were shown in the patient with the TNFSF13B mutation. Prevalence of heterozygous SLC52A3 BVVL causative mutations and notable variability in expressivity of homozygous and heterozygous genotypes are being reported for the first time. Identification of WDFY4 and TNFSF13B as candidate causative genes supports conjectures on involvement of the immune system in BVVL and amyotrophic lateral sclerosis.

Sex Differences in 28-Day Mortality of Ischemic Stroke in Iran and Its Associated Factors: A Prospective Cohort Study.

INTRODUCTION: The mortality and morbidity rates of stroke in men and women have been reported differently and its effective factors have been discussed. The purpose of this study was to investigate sex differences in 28-day mortality of ischemic stroke and its associated factors. MATERIALS AND METHODS: This is a prospective cohort study conducted from June 2018 to September 2019 in patients with ischemic stroke referred to Firoozgar, Shariati and Sina hospitals in Tehran. Demographic data, risk factors, disease history, drug use, severity of stroke, and patient functional status were recorded in the hospital. The patients' functional status and severity of stroke were measured using the Modified Ranking Scale (MRS) and the National Institutes of Health Stroke Scale (NIHSS). After 28 days, the patients' survival status was monitored. Logistic regression was used to analyze the data. RESULTS: In this study, 703 patients were enrolled; of them, 260 (37.00%) were female and 443 (63.00%) were male. After 28 days, 21 female cases (8.17%) and 26 male (6.08%) ones died (P = 0.299). Functional status (OR = 4.65; 95%CI: 2.09 to 10.38), diastolic blood pressure (OR = 0.91; 95%CI: 0.85 to 0.96), warfarin use (OR = 0.15; 95%CI: 0.04 to 0.55), and hemoglobin (OR = 1.17; 95%CI: 1.02 to 1.35) were associated with 28-day mortality. Poor functional status in men had a greater association with 28-day mortality than women (OR 4.65 vs. 1.64). High diastolic blood pressure had a negative association with the 28-day mortality of cases and this association is more in women than in men (OR 0.88 vs. 0.91). High hemoglobin is a risk factor in men and a protective factor in 28-day mortality in women (OR 1.73 vs. 0.73). Smoking also had a greater association with 28-day mortality in women than men (OR 2.67 vs. 1.2). DISCUSSION: Twenty eight-day mortality was more in women than in men, but this difference was not significant. Women were older, had more severe stroke and poorer functional status than men. Variables including functional status, diastolic blood pressure, hemoglobin level, and smoking had interaction with sex, and their association with 28-day mortality rate was different between men and women. Sex differences should be considered, so that we can better manage stroke patients.

Guillain-Barre syndrome in patients with coronavirus disease-2019: Report of six cases and review of literature.

Background: Few studies have reported the association of Guillain-Barre syndrome (GBS) and coronavirus disease-2019 (COVID-19) infection. In this study, we reported GBS in six patients infected with COVID-19 and reviewed all existing literature about GBS in association with COVID-19. Methods: This study was performed in three referral centers of COVID-19 in Iran, and six patients with the diagnosis of GBS were enrolled. Patients enrolled in the study with acute progressive weakness according to the demyelinating or axonal variant of GBS, according to Uncini's criteria. Results: Four of our patients had axonal polyneuropathy, two patients had demyelinating polyneuropathy, and one patient required mechanical ventilation. All our patients had a favorable response to treatment. In one patient, the GBS symptoms recurred four months after the first episode. Conclusion: Limited case reports suggest a possible association between GBS and COVID-19. Such associations may be an incidental concurrence or a real cause-and-effect linkage; however, more patients with epidemiological studies are necessary to support a causal relationship.

The use of brain stimulation in the rehabilitation of walking disability in patients with multiple sclerosis: A randomized double-blind clinical trial study.

Background: Transcranial direct current stimulation (tDCS) of the primary motor cortex of the lower limb has been exploited in the treatment of patients with stroke and spastic lower limb paresis. We examined this stimulation efficacy in the treatment of multiple sclerosis (MS)-related walking disability. Methods: In a single-center randomized double-blind clinical trial study, 13 patients with MS and walking disability and Expanded Disability Status Scale (EDSS) score of 3 to 6 were randomized to the real and sham stimulation groups. In the real tDCS stimulation, 7 patients received anodal 2.5 mA stimulation at 1 cm anterior to the Cz point for 30-minute daily sessions in 7 consecutive days. The other group received sham stimulation with the same protocol. The primary outcome of the trial was change in the Timed 25-Foot Walk (T25-FW) from before to after the stimulation. We also assessed the Multiple Sclerosis Walking Scale-12 (MSWS-12). We employed linear mixed effects model to examine the efficacy of tDCS stimulation on changing the outcomes. Results: On average, patients who received real tDCS stimulation walked faster after 7 sessions of stimulation [Estimate = -2.7, standard error (SE) = 1.3, P = 0.049], while walking speed of sham stimulation recipients did not change. For every session of stimulation, recipients of real tDCS stimulation spent 2.7 seconds less for walking the 25 feet. Real tDCS stimulation was not effective in improving MSWS-12 scores. Conclusion: tDCS stimulation of the lower limb motor cortex speeded up patients with MS in walking, but without improvement in patients' mobility in daily activities.

Elevated troponin T after acute ischemic stroke: Association with severity and location of infarction.

BACKGROUND: Serum troponin elevation, characteristic of ischemic myocardial injury, has been observed in some acute ischemic stroke (AIS) patients. Its cause and significance are still controversial. The purpose of this study is to find determinants of troponin elevation and its relationship with stroke severity and location. METHODS: Between January 2013 and August 2013, 114 consecutive AIS patients confirmed by diffusion-weighted magnetic resonance imaging were recruited in this study. Serum troponin T level was measured as part of routine laboratory testing on admission. Ten lead standard electrocardiogram (ECG) was performed and stoke severity was assessed based on National Institutes of Health Stroke Scale (NIHSS). RESULTS: Troponin T was elevated in 20 (17.6%) of 114 patients. Patients with elevated troponin were more likely to have higher age, higher serum creatinine and ischemic ECG changes. Troponin levels were higher in patients with more severe stroke measured by NIHSS [7.96 (6.49-9.78) vs. 13.59 (10.28-18.00)]. There was no association between troponin and locations of stroke and atrial fibrillation. There were 6 (5%) patients with elevated troponin in the presence of normal creatinine and ECG. CONCLUSION: Stroke severity, not its location, was associated with higher troponin levels. Abnormal troponin levels are more likely, but not exclusively, to be due to cardiac and renal causes than cerebral ones.