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Myasthenia gravis (MG) is an immune-mediated potentially treatable disease in which rapid diagnosis and proper treatment can control symptoms. Treatment should be individualized in each patient according to distribution (ocular or generalized) and severity of the weakness, antibody status, thymus pathology, patient comorbidities, and preferences. A group of Iranian neuromuscular specialists have written these recommendations to treat MG based on national conditions. Four of the authors performed an extensive literature review, including PubMed, EMBASE, and Google Scholar, from 1932 to 2020 before the central meeting to define headings and subheadings. The experts held a 2-day session where the primary drafts were discussed point by point. Primary algorithms for the management of MG patients were prepared in the panel discussion. After the panel, the discussions continued in virtual group discussions, and the prepared guideline was finalized after agreement and concordance between the panel members. Finally, a total of 71 expert recommendations were included. We attempted to develop a guideline based on Iran’s local requirements. We hope that these guidelines help healthcare professionals in proper treatment and follow-up of patients with MG.

Purpose: We evaluated the ability of muscle ultrasound (MUS), a non-invasive and simple tool, to distinguish between healthy subjects and individuals with inflammatory myopathy. Methods: This study was conducted on 17 patients with recently diagnosed biopsy-proven inflammatory myopathies (12 dermatomyositis, five polymyositis) compared with 17 age- and gender-matched healthy control adults. All patients underwent clinical assessments, including manual muscle testing (MMT) and hand-held dynamometry, as well as MUS evaluations, including thickness and echointensity (EI) in predefined muscle groups. Results: The disease duration was seven months (interquartile range: 3-11). Except for biceps and gastrocnemius, the patients' muscles had significantly higher EI and lower thickness in comparison with the controls, The EI sum-score manifested the highest area under curve (AUC) in comparison with the sum-scores of other variables (EI vs. MMT: AUCs-difference = 0.18, p < 0.01; EI vs. dynamometry: AUCs-difference = 0.14, p = 0.02; EI vs. thickness: AUCs-difference = 0.25, p < 0.01). Conclusion: EI of muscles differed significantly between healthy individuals and those with inflammatory myopathies and may potentially serve as a useful diagnostic biomarker.

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.

The Article Abstract is not available.

Background: This retrospective cohort study was conducted to evaluate the efficacy and tolerance of rituximab (RTX) for the management of myasthenia gravis (MG). Methods: This retrospective cross-sectional study was conducted on 61 patients with refractory and non-refractory MG who received RTX. The Myasthenia Gravis Activities of Daily Living (MG-ADL) profile was used to assess MG symptoms and their effects on daily activities at the start of RTX and in the last follow-up. The Myasthenia Gravis Foundation of America Post-Intervention Status (MGFA-PIS) scale has been used as an outcome measure after treatment with RTX in the 12th month and the last follow-up. Results: The mean age of the patients was 40.31 ± 13.53 years (range: 15-78 years). Of 61 patients, eight (13.1%) were double seronegative, 29 (47.5%) had anti-acetylcholine receptor (AChR+) antibody, and 24 (39.3%) had anti-muscle-specific tyrosine kinase antibody (MuSK+). According to the mean rank table, the results of this study showed that the drug was more effective in improving the symptoms of MuSK+ patients compared to the other two groups (P = 0.006). The mean MG-ADL was 4.86 ± 1.83 before treatment and 1.51 ± 2.02 in the last follow-up visit. Paired t-test showed a significant association between MG-ADL before and after treatment in the last visit [t(55): 11.30, 95% confidence interval (CI): 2.79-3.99, P = 0.001)]. Conclusion: This retrospective study showed a considerable effect of RTX as induction therapy in patients with MG, especially those with MuSk+ MG.

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.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.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.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.

Aims We aimed to assess the compound muscle action potential (CMAP) scan in the follow-up of chronic inflammatory demyelinating polyneuropathy (CIDP) patients and investigate the correlation of CMAP scan parameters with functional and standard electrodiagnostic tests. Methods We evaluated four parameters of abductor pollicis brevis (APB) CMAP scan (i.e., step numbers, step percentage, S10, S90), functional measures (e.g., Medical Research Council Sum Scores), and electrodiagnostic tests, including nerve conduction study (NCS) and motor NCS of the median nerve in the baseline and after six months of treatment. Results Twenty patients completed baseline clinical and electrodiagnostic studies. However, sixteen patients completed the follow-up study. The median of step numbers at baseline was 3.5 (2–4.2), which decreased to 2.5 (0–3) (p = 0.005). After the treatment, step percentage reduced from 28.6 (23.9–38.7) to 13.4 (0–23.6) (p = 0.001). The scores obtained from the clinical scales showed significant recovery of most of the functions, while the alterations of NCSS and NCS of the median nerve were not significant. Conclusions We found a significant reduction in step number and step percentage after follow-up. This alteration was not reflected in standard electrodiagnostic values. The improvement of functional scales alongside the CMAP scan parameters suggests that the CMAP scan could be considered an appropriate outcome measurement in research and clinical fields.

Opium sale is prohibited in Iran, and sellers mix it with lead to increase the weight. Herein, we describe neuromuscular findings in nine patients who developed lead poisoning due to opium/drug consumption. The mean age of patients was 53 ± 3.5 years and their mean blood lead level was 122.6 ± 20.8 μg/dL. Most of the patients had an acute/subacute course presenting as axonal or demyelinating sensorimotor polyneuropathy, motor neuron disease, and multiple mononeuropathies with favorable response to the chelation therapy. Lead poisoning should be considered in patients with a history of opium use and neuromuscular symptoms.

OBJECTIVES In addition to the physical disability in chronic inflammatory demyelinating polyneuropathy (CIDP), various aspects of quality of life (QoL) are affected by the disease. Our goal was to evaluate the QoL in Iranian patients with CIDP and to study the association of clinical and demographic factors with QoL parameters. METHODS Twenty-six Iranian patients with CIDP were asked to complete a standardized Persian version of SF-36, and the association of demographic and functional parameters with QoL parameters was assessed. RESULTS Sex, living place, and income had no significant effect on QoL. Mean physical composite score was 37.7 ± 9.4, and mean mental composite score was 37.2 ± 15.2 that was lower than similar studies. There was a positive correlation between QoL and muscle strength of different muscle groups [hand (r = 0.41, P = 0.04); hip (r = 0.44, P = 0.02); and foot (r = 0.41, P = 0.04); total manual muscle testing (r = 0.46, P = 0.02)] and a significant negative correlation between the duration of illness and mean physical composite score (r = -0.61, P = 0.00). CONCLUSIONS Our patients achieved low scores in both the physical and mental aspects of QoL compared with other studies. We found a significant correlation between the physical domain of the SF-36 score and muscle strength of the hand, hip, and foot muscle group and with total manual muscle testing, suggesting that physical disability is the most crucial factor affecting QoL. Also, there was a significant negative association between the duration of the disease and the physical domain.

Objective outcome measures to monitor treatment response and guide treatment are lacking in chronic inflammatory demyelinating polyneuropathy (CIDP). In this study we aimed to evaluate the motor unit number index (MUNIX) as an outcome measurement in patients with CIDP and determine the correlation of MUNIX with functional and standard electrodiagnostic tests in a single follow-up study.We evaluated MUNIX of the abductor pollicis brevis, abductor digiti minimi, and tibialis anterior (TA) muscles bilaterally. Muscle force was assessed by Medical Research Council Sum Score (MRCSS). Functional measures used were the Overall Neuropathy Limitation Score (ONLS) and the Rasch-built Overall Disability Scale (R-ODS) score at baseline and after 6 months of treatment. Standard electrophysiology was evaluated by the Nerve Conduction Study Score (NCSS).Twenty patients were included at baseline, and 16 completed the follow-up study. Significant correlations were found between the MUNIX sum score and both MRCSS and NCSS at baseline, between both the pinch strength and grip and upper limb MUNIX at baseline and follow-up, and between MUNIX of TA and both lower limb MRCSSs with lower limb ONLS at baseline and follow-up. Significant correlations also were found between MUNIX sum score change and MRCSS change, R-ODS change, and ONLS change.MUNIX changes correlated with strength and electrophysiological improvements in CIDP patients. This suggests that MUNIX may represent a useful objective biomarker for patient follow-up.

The follow-up and monitoring of response to immunomodulatory therapy in patients with chronic inflammatory demyelinating polyneuropathy are still challenging. Various outcome measures have been proposed in recent years, and some are now frequently used in daily clinical practice; however, reliable biomarkers for the disease activity and treatment response are lacking.Cross-sectional nerve area of the bilateral vagus, fifth and the sixth cervical spinal, median, ulnar, tibial, peroneal, and sural nerves were measured at 2 time points with an interval of 6 months using nerve ultrasound. The results were used to calculate the ultrasound pattern sumscore (UPSS). The correlation between UPSS change (ΔUPSS) and changes in functional and nerve conduction studies measures over the study period were assessed.Sixteen patients completed this prospective, observational study. General linear model showed that ΔUPSS is significantly associated with ΔMedical Research Council sumscore (β = -0.72, P = 0.003), Δhandgrip strength (β = -0.57, P = 0.014), ΔRasch-built overall disability scale (β = -0.57, P = 0.010), and Δoverall neuropathy limitations scale (β = 0.75, P0.001), after adjustment of confounding variables. Nevertheless, ΔUPSS was not correlated with other clinical measures, including Δpinch power, Δ9-hole peg test, Δ10-m walking test, and Δnerve conduction study sumscore (P values0.05).Nerve ultrasound might be an efficient method for monitoring the functional status of patients with chronic inflammatory demyelinating polyneuropathy over time because the alterations in its scores could significantly reflect clinical changes.

Background and aims Few case reports/series describe the efficacy of rituximab in refractory CIDP, which is preferred in the presence of anti-nodal/paranodal antibodies. We aimed at evaluating the clinical response to rituximab in a subset of patients with refractory CIDP for whom anti-nodal/paranodal antibodies status was unknown, as not available in Iran. Methods We retrospectively analyzed the response to rituximab in 14 Iranian patients with refractory CIDP (3 children, 11 adults), in whom the anti-nodal/paranodal antibodies status was unknown. Subjects were evaluated with the Medical Research Council (MRC) sum score (MRCSS), Inflammatory Neuropathy Cause and Treatment (INCAT) disability scores, and electrophysiology, before and after treatment. Results Mean age was 34.4 ± 20.7 years, disease duration pre-rituximab treatment was 27.8 ± 18.8 (range: 6 - 60) months, and mean follow-up duration was 18.5 ± 11.0 (range: 4 - 36) months. Considering INCAT sumscore, one worsened during post-rituximab treatment, and three patients did not change. Considering MRCSS, notably, four patients achieved normalization of their MRCSS. Regarding corticosteroid dose, two patients could discontinue prednisolone. As rated by a pre-defined scoring system, nerve conduction parameters improved significantly post-rituximab in the treated cohort (p=0.006). All patients tolerated rituximab infusions without adverse effects. Interpretation Rituximab may be effective in refractory CIDP, even though worsening may occur in some patients. Anti-nodal/paranodal antibodies assay, when available, and other criteria may help drive therapeutic decision-making on rituximab as second-line treatment. This article is protected by copyright. All rights reserved.

Background: Migraine is a chronic medical problem and sometimes progressive disorder characterized by recurrent episodes of headache. Nutritional factors can reduce and prevent the severity and frequency of migraine. Objectives: This study aimed to assess the relationship between major dietary patterns and disease severity among migraine patients. Methods: This cross-sectional study included 266 females (18 - 50 years old) who attend neurology clinics of Sina and Khatam Alanbia hospitals, and a professional headache clinic, both in Tehran, Iran, for episodic migraine diagnosis in 2016. The participants’ data was gathered using a general questionnaire and medical history. Dietary intake was assessed using a 147-item semi-quantitative food frequency questionnaire (FFQ). Anthropometric measurements were taken for all cases. visual analog scale (VAS) and migraine disability assessment (MIDAS) questionnaires were used by a neurologist for assessing migraine disability and pain severity, respectively. Also, principal component analysis (PCA) was used to identify major dietary patterns. The association between dietary patterns and disease severity was evaluated using multinomial logistic regression. Results: Using the PCA, two major dietary patterns, including the healthy and unhealthy diet, were identified. More adherence to the healthy dietary pattern (high in fruits, fruit juices, and dried fruits, vegetables, whole grains, liquid oil, brains, beans, low-fat dairy, and white meat) was associated with a lower VAS score after adjusting for energy intake, BMI, water intake, and increased salt intake (OR = 1.82, 95% CI = 0.96 - 3.44, P-trend < 0.005). The intensity of migraine headache increased by 82% in the lowest adherence to this pattern compared to the most adherence. There was no significant association between healthy and unhealthy dietary patterns with MIDAS before and after controlling for confounding variables. Conclusions: This study showed that adherence to a healthy dietary pattern reduces the severity of pain in migraine patients. However, further studies are needed to evaluate the relationship between the unhealthy dietary pattern and the severity of migraine symptoms.

Background: Caregivers of patients with amyotrophic lateral sclerosis (ALS) may suffer from anxiety, depression, and reduced quality of life (QoL). Our goal was to evaluate the QoL and mood disorders in caregivers and their correlation with the patients' demographic, physical, and mental conditions. Methods: We analyzed data from 39 patients with ALS and their caregivers. Patients completed questionnaires of anxiety assessed by Generalised Anxiety Disorder Assessment (GAD-7), depression using the Beck Depression Inventory-II (BDI-II), and QoL via 40-item Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40). Physical impairment was also measured in the patients using the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R). Caregivers were also assessed by BDI-II, GAD-7, and 36-item Short-Form Health Survey questionnaire (SF-36). Results: The prevalence of depression and anxiety in the patients was 82.1% and 71.8%, respectively. Caregivers also had higher rates of anxiety and depression and lower levels of QoL in comparison with the general population (anxiety: 66.7%, depression: 43.6%). Depression and anxiety were considerably associated with worsened QoL in the caregivers. None of the demographic, physical, or mental characteristics of patients with ALS were related to either mood status or QoL of the caregiver population. Conclusion: Caregivers experience higher rates of anxiety and depression and lower QoL in comparison with the general population. The severity of mood disorders is inversely associated with the physical and mental domains of caregivers' QoL. Nonetheless, QoL in the caregivers is not affected by the physical or mental disability of the patients.

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.

Several neurological presentations have been reported following coronavirus 2019 (COVID-19) infection. This case report describes three myasthenia gravis (MG) patients presented following COVID-19 infection. We report three adult patients with myasthenic Gravis and COVID-19 infection. The patients are between 38 and 61 years old. Case 1 is a 61-year-old woman with progressive dysphagia, nasal speech, ocular ptosis, diplopia, and proximal muscle weakness for 10 days. She had a COVID-19 infection 6 weeks ago. Case 2 is a 57-year-old man with clinical symptoms of muscular fatigability, diplopia, ptosis, and dysphagia for a week and a positive COVID-19 infection 10 days ago. Case 3 is a 38-year-old woman with fatigability, ptosis, dysphagia, and a diagnosis of COVID-19 infection 4 weeks ago. All patients had a positive RT-PCR for COVID-19 infection by nasopharyngeal swab test and a high-level acetylcholine receptor antibody in the serum. All patients were treated with pyridostigmine and prednisolone with a favorable outcome. MG may appear following COVID-19 infection, and the role of molecular mimicry and latent MG activation should be considered the cause of the disease onset., Graphical Abstract ga1

The article's abstract is not available.

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.

Background and purpose Very-long-chain acyl-CoA dehydrogenase deficiency (VLCADD) is a hereditary disorder of mitochondrial long-chain fatty acid oxidation that has variable presentations, including exercise intolerance, cardiomyopathy and liver disease. The aim of this study was to describe the clinical and genetic manifestations of six patients with adult-onset VLCADD. Methods In this study, the clinical, pathological and genetic findings of six adult patients (four from Iran and two from Serbia) with VLCADD and their response to treatment are described. Results The median (range) age of patients at first visit was 31 (27-38) years, and the median (range) age of onset was 26.5 (19-33) years. Parental consanguinity was present for four patients. Four patients had a history of rhabdomyolysis, and the recorded CK level ranged between 67 and 90 000 IU/l. Three patients had a history of exertional myalgia, and one patient had a non-fluctuating weakness. Through next-generation sequencing analysis, we identified six cases with variants in the ACADVL gene and a confirmed diagnosis of VLCADD. Of the total six variants identified, five were missense, and one was a novel frameshift mutation identified in two unrelated individuals. Two variants were novel, and three were previously reported. We treated the patients with a combination of L-carnitine, Coenzyme Q10 and riboflavin. Three patients responded favorably to the treatment. Conclusion Adult-onset VLCADD is a rare entity with various presentations. Patients may respond favorably to a cocktail of L-carnitine, Coenzyme Q10, and riboflavin.

Contains fulltext : 229728.pdf (Publisher’s version ) (Closed access) INTRODUCTION: We evaluated the association between muscle ultrasound, number of motor units, and clinical parameters, and assessed their utility for distinguishing amyotrophic lateral scleorisis (ALS) patients from healthy individuals. METHODS: Three muscle pairs (abductor pollicis brevis, abductor digiti minimi, and tibialis anterior) of 18 ALS patients and 18 controls underwent muscle ultrasound (echointensity and thickness) and assessment of motor unit number index (MUNIX). The clinical and functional status of participants were also assessed. RESULTS: Mean age of the patients was 53.8 ± 12.1 years, and score on the ALS Functional Rating Scale-Revised was 38.9 ± 4.1. Echointensity of all tested muscles of ALS participants was significantly higher than that of controls, but there was no significant difference in muscle thickness. Muscle echointensity correlated significantly with clinical and electrophysiological parameters. CONCLUSION: Echointensity of muscles was highly associated with clinical scales and MUNIX, confirming its relevance as an ancillary diagnostic test in ALS patients.

Purpose/introduction: Migraine is a common disorder, with attacks causing neurological dysfunction and pain. Many foods are involved in reducing the severity of migraine attacks. This study aimed to assess the effects that adhering to the Dietary approaches to stop hypertension (DASH) diet had on headache severity and duration among women suffering from migraine.Methods and materials: Two hundred and sixty-six women (18-45 years) were enrolled after being referred to a headache clinic for the first time. Dietary intake was assessed daily using a Food Frequency Questionnaire. Anthropometric measurements were assessed for all cases, as well as headache duration of each attack; Visual Analog Scale and Migraine Disability Assessment questionnaires were evaluated by a neurologist.Results: The mean age, weight, and height of the study participants were 34.32 (SD 7.86) years, 69.41 (13.02) kg, and 161 (0.05) cm, respectively. The results of analysis in the crude model showed that individuals with the greatest adherence to the DASH diet displayed a 30% lower prevalence in severe headaches, compared to those with the lowest adherence (OR=0.70, 95%CI=0.49-0.99, P

Background. The mechanism of stroke in beta-thalassemia was reported previously as cardioembolic and hypercoagulable state. However, there is no report of watershed infarct in beta-thalassemia anemia.Method. We present an adultβ-thalassemia major patient with manifest asymptomatic chronic left carotid occlusion who suffered watershed infarct.Result. In the presence of asymptomatic chronic left internal carotid occlusion, we assumed that severe anemia (hemoglobin = 3) at admission leads to watershed infarct.Conclusion. Watershed infarct seems to be the cause of stroke in cases ofβ-thalassemia major with severe anemia. Blood transfusion can be applied in the setting of acute brain ischemia in such high risk patients.

The article's abstract is not available.

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Migraine is a common chronic inflammatory neurological disease with the progressive and episodic course. Much evidence have shown a role of inflammation in the pathogenesis of migraine. Omega-3 fatty acids are an important components of cell membranes phospholipids. The intake of these fatty acids is related to decrease concentration of C-reactive protein (CRP), proinflammatory eicosanoids, cytokines, chemokines and other inflammation biomarkers. Many of clinical trials have shown the beneficial effect of dietary supplementation with omega-3 fatty acids in inflammatory and autoimmune diseases in human, including Parkinson’s disease (PD), amyotrophic lateral sclerosis (ALS), Alzheimer’s disease (AD), multiple sclerosis (MS) and migraine headaches. Therefore, omega-3 fatty acids as an alternative therapy can be potentially important. This review focuses on the pathogenesis of a migraine, with an emphasis on the role of omega-3 fatty acid and its molecular mechanisms.

A 23-year-old woman was admitted to the emergency department with the history of headache, serial seizures, and decreased the level of consciousness from a week before. At admission, blood pressure was 230/170 mmHg, and creatinine level was 7.6 mg/dl. Initial brain and cervical magnetic resonance imaging (MRI) revealed hyperintense lesions on fluid-attenuated inversion recovery (FLAIR) in bilateral occipital lobes and a longitudinally extensive lesion in the spinal cord (Figure 1, A-C). In the laboratory investigations, the level of anti-double stranded DNA was 45 IU/ml (normal < 10 IU/ml) and anti-nuclear antibody titer was high (> 1/160). Moreover, in renal biopsy, lupus nephritis was reported. Two weeks later, after hypertension treatment, the hyperintense signals wholly disappeared (Figure 1, D-F). Figure 1. Axial fluid-attenuated inversion recovery (FLAIR) brain magnetic resonance imaging (MRI) indicating hypersignal lesions in parieto-occipital areas in favor of posterior reversible encephalopathy syndrome (PRES) at admission (A, B); sagittal T2 cervical MRI demonstrating a longitudinally extensive lesion in the spinal cord at admission (C); two weeks later, the hyperintense signals had completely disappeared on the brain and spinal cord MRIs (D-F).

Migraine is a destabilizing neuroinflammatory disorder characterized by recurrent headache attacks. Evidences show tumor necrosis factor (TNF)-α play a role in neuroimmunity pathogenesis of migraine. TNF-α increase prostanoid production, hyperexcitability of neurons, and nociceptor activation resulted in neuroinflammation and neurogenic pain. ω-3 fatty acids and curcumin exert neuroprotective and anti-inflammatory effects via several mechanisms including suppression of TNF-α gene expression and its serum levels. The aim of this study is an evaluation of synergistic effects of ω-3 fatty acids and nano-curcumin on TNF-α gene expression and serum levels in migraine patients. The present study performed as a clinical trial over a 2 month period included 74 episodic migraine patients in 4 groups and received ω-3 fatty acids, nano-curcumin, and combination of them or placebo. At the start and the end of the study, the gene expression of TNF-α and TNF-α serum levels was measured by real-time PCR and ELISA method, respectively. Our results showed that the combination of ω-3 fatty acids and nano-curcumin downregulated TNF-α messenger RNA (mRNA) significantly in a synergistic manner (P

Acute onset bulbar symptoms with respiratory failure and descending paralysis may occur in several neuromuscular disorders including variants of Guillain-Barre syndrome (GBS), diphtheria, botulism and toxins. We present a 51-year-old man who presented with complains of ptosis and dyspnea following pyrethroids spraying in an enclosed area for eradication of flea. Within 5-6 days of admission limb weakness, dysphagia, dysarthria, blurred vision, diplopia, tremor and respiratory distress added to previous symptoms. Temporal profile of events after exposure, development of similar symptoms in patient's son, electrodiagnostic findings and exclusion of other etiologies confirms intoxication etiology. We reviewed the literature and provide an extensive electrodiagnostic overview.

Background: Guillain-Barre syndrome (GBS) is an immune-mediated peripheral neuropathy. We compared clinical, laboratory characteristics, and disease course of GBS subtypes in a large group of Iranian patients in Isfahan. Materials and Methods: We collected data from patients who were admitted to Alzahra referral university Hospital, Isfahan, Iran with a diagnosis of GBS. In this population-based cross-sectional research, characteristic of 388 cases with GBS between 2010 and 2015 were studied. Results: The current study recruited 388 patients with GBS including 241 males (62.1%) and 147 females (37.9%) with a mean age of 42.78 ± 21.34. Patients with polyradiculopathy had the highest mean age of 55.12 ± 20.59 years, whereas the least age was seen in acute motor axonal neuropathy (AMAN) with the mean of 36.30 ± 18.71 years. The frequency of GBS witnessed the highest frequency in spring with 113 cases (29.1%) and winter with 101 cases (26%). Patients' electrodiagnostic findings indicated that the highest frequency pertained to AMSAN with 93 cases (24%), whereas the least frequent diagnosis was acute Polyradiculopathy with 8 cases (2.1%). Most of the patients did not have any infections (53.6%) and among patients with infections, AMSAN had the highest frequency (22.9%) and finally, patients with AMSAN and AMAN had a higher length of stay. Conclusion: The study demonstrated incidence, sex distribution, preceding infection, and surgery similar to previous studies. However, our data differs from a study in Tehran that showed acute inflammatory demyelinating polyradiculoneuropathy is more prevalent than other types and we found a seasonal preponderance in cold months, particularly in axonal types.

Introduction: Moyamoya disease (MD) is a disorder caused by blocked arteries at the base of the brain. As the normal blood vessels narrow and become blocked, a person may suffer a stroke. The MD mainly affects children, although adults may have the condition. This is a rare condition, with a marked prevalence gradient between Asian countries. Case Presentation: We report a 5-year-old boy, who was presented with sudden right hand monoparesis and dysarthria. Conclusions: The patient was investigated with magnetic resonance angiography and diagnosed as MD.

To determine the association between air pollution and vitamin D status in young children living in a sunny region.This cross-sectional study of 100 children aged 4-10 years in Isfahan was conducted during 2011-2012. This industrial city has an arid climate and is the second most air-polluted city in Iran. Children were selected by random cluster sampling from various areas with different levels of air pollution. The air quality index (AQI) was recorded and demographic variables, dietary habits and levels of physical activity were determined by validated questionnaires. Serum concentrations of 25-hydroxyvitamin D [25(OH)D], calcium, phosphate, alkaline phosphatase and parathyroid hormone were measured. The P for trend for median (interquartile range) of variables was assessed across the AQI quartiles. The associations between AQI and biochemical values were assessed by multiple linear regression after adjustment for age, gender, BMI, diet and pattern of physical activity.Ninety-seven children (46.4% boys) completed the study. The median (interquartile range, IQR) of serum 25(OH) D was 37.4 (22.5, 81.6) nmol/L. Median dietary vitamin D intake was 11.7 μg/day, i.e. 78% of the required daily amount. Vitamin D deficiency and insufficiency were detected in 37.9% and 46.3% of children, respectively. Median (IQR) exposure to ultraviolet B radiation in the AQI Quartile 4 area was significantly lower than in the Quartile 1 area [0.36 (0.35-0.38) watts per square metre (W/m(2)) vs 0.41 (0.39-0.44) W/m(2), respectively, P = 0.04]. Likewise, the corresponding figure for serum 25(OH)D was 35.2 (22.5-45.2) vs 52.7 (44.9-81.6) nmol/L, respectively, P = 0.04. AQI was inversely associated with serum 25(OH)D, which remained significant after adjustment for the above-mentioned confounding factors (β = 0.61, P = 0.01).The independent inverse association of AQI with 25(OH)D explains the high prevalence of vitamin D deficiency in children in Isfahan. Dietary intake of vitamin D was not sufficiently low to explain the very low level of serum 25(OH)D. In regions with plenty of sunlight, air pollution should be considered to be a factor in the causation of hypovitaminosis D.

The autoimmune disease myasthenia gravis (MG), can mimic a variety of neurological disorders leading to a delay in diagnosis and treatment. On occasions, misdiagnosis of MG could lead to unnecessary therapeutic interventions. We report the case of a 50 year-old man, in whom MG was mistaken for motor neuron disease (MND). Subsequently, correct diagnosis and optimal management resulted in saving his life and significant improvement in his functional status. We discuss the importance of considering MG as one of the potential differential diagnoses among cases of new onset or recurrent unexplained bulbar symptoms, despite exaggerated deep tendon reflexes. Also, a literature review on the misdiagnosis of MG and the potential pitfalls in MG diagnosis are discussed.