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.

Iranian Consensus Recommendations for Treatment of Myasthenia Gravis.

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.

Disease severity and response to treatment in Iranian patients with myasthenia gravis.

PURPOSE: Myasthenia gravis (MG) is a potentially fatal neuromuscular disorder if left untreated. In this study, we tried to address the possible demographic, clinical, and laboratory determinants of severity and outcome in Iranian MG patients over a follow-up period of more than 5 years. METHODS: Demographic and diagnostic data (age, age of onset, antibody status, thymus pathology, and duration of the disease) of the patients with MG were extracted. Maximal disease severity and post-intervention status were assessed according to the recommendations of the task force of the Myasthenia Gravis Foundation of America. RESULTS: In our series of 146 patients, MG was more severe in older, anti-muscle specific tyrosine kinase (MuSK) positive, and thymomatous patients. Seropositivity to the MuSK antibody and the presence of thymoma determined the need for immunosuppressive drugs. However, the number of patients requiring more than one immunosuppressive was not significantly different among various subtypes. CONCLUSIONS: The overall outcome was favorable in the majority of patients, despite differences in the disease course and severity. In contrary to the previous reports, anti-MuSK positive patients in our series did not need a more vigorous treatment regimen comparing other serologic subtypes of MG.

Association of HLA Class II Alleles with Disease Severity and Treatment Response in Iranian Patients with Myasthenia Gravis.

Myasthenia gravis is an autoimmune neuromuscular disease with a multifactorial etiology. A major part of the genetic susceptibility belongs to the HLA encoding genes. In this study, we investigated the role of HLA class II polymorphism in disease severity, and treatment response. In our 146 patients, 15 DRB1, 7 DQA1, and 9 DQB1 alleles, and 19 haplotypes were found. Adjusted p-values did not show any significant associations between these loci, disease severity and treatment outcome. Further studies in different populations with a larger number of patients are needed to determine the exact contribution of HLA class II alleles to MG prognosis.

Investigating the possible association between NLRP3 gene polymorphisms and myasthenia gravis.

INTRODUCTION: In this case-control study, we investigated the association between nucleotide oligomerization domain-like receptor family pyrin domain containing 3 (NLRP3) single-nucleotide polymorphisms (SNPs) rs10754558, rs3806265, rs4612666, and rs35829419 and myasthenia gravis (MG). METHODS: Samples from MG patients were selected from a previous study conducted in our neuromuscular clinic, which investigated the association between human leukocyte antigen (HLA) class II genes and MG. Genetic data of controls were also available from another study. The NLRP3 SNPs genotyping was performed using the TaqMan method. RESULTS: A total of 93 blood samples from eligible Iranian patients with MG and 56 samples from healthy controls were obtained. The NLRP3 rs3806265 "C" allele was significantly more frequent in MG patients (P < .001; odd ratio [OR] = 2.33, 95% confidence interval [CI]: 1.4-4.0) than controls. The "CC" genotype of this SNP was found in 18.27% of patients, but none of the controls (P < .001). The distribution of other SNPs was similar between the groups. DISCUSSION: These preliminary results suggest that there might be some associations between the NLRP3 gene polymorphism and MG.

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.

Distinct Clinical and Genetic Findings in Iranian Patients With Glycogen Storage Disease Type 3.

OBJECTIVES: Glycogen storage disease type 3 (GSD-III) is a rare inherited metabolic disorder caused by glycogen debranching enzyme deficiency. Various pathogenic mutations of the AGL gene lead to abnormal accumulation of glycogen in liver, skeletal, and cardiac muscles. Here, we report distinct clinical and genetic data of Iranian patients with GSD-III. METHODS: Clinical and laboratory data of 5 patients with GSD-III were recorded. Genetic investigation was performed to identify the causative mutations. RESULTS: Three patients had typical liver involvement in childhood and one was diagnosed 2 years after liver transplantation for cirrhosis of unknown etiology. Four patients had vacuolar myopathy with glycogen excess in muscle biopsy. All patients had novel homozygous mutations of the AGL gene namely c.378T>A, c.3295T>C, c.3777G>A, c.2002-2A>G, and c.1183C>T. CONCLUSIONS: This is the first comprehensive report of patients with GSD-III in Iran with 2 uncommon clinical presentations and 5 novel mutations in the AGL gene.

Olfactory Loss Management in View of Avicenna: Focus on Neuroprotective Plants.

BACKGROUND: Loss of olfaction can cause noticeable reduction in general quality of life. Only a small portion of patients with olfactory loss respond to current medications. Thus, development of novel therapeutic strategies seems to be necessary. Looking into traditional medical knowledge can be of great value in addressing useful remedies. Traditional Persian Medicine (TPM) has been practiced in Persia for more than 2000 years. Avicenna is the most eminent Persian physician. OBJECTIVE: To survey Avicenna's views on etiology and management of olfactory loss and to search for relevant modern pharmacological data. METHODS: Avicenna's views on etiology and management (including suggested medicinal plants) of olfactory loss were obtained from "Canon of Medicine". In addition, a detailed search in ScienceDirect, PubMed, Scopus and Google Scholar databases was performed to elucidate relevant pharmacological actions and mechanisms of the plants and their major compounds with special focus on neuroprotective activity. RESULTS: Acorus calamus L., Allium cepa L., Allium sativum L., Aloe spp., Cinnamomum cassia (L.) J.Presl, Lavandula stoechas L., Mentha longifolia (L.) L., Nigella sativa L., Peganum harmala L., Piper nigrum L. and Zingiber officinale Roscoe were found to be the most emphatic plants for the treatment of olfactory loss. Pharmacological studies revealed biological activities including neuroprotective, anti-inflammatory, free radical scavenging activities and promoting endogenous antioxidant capacity for these plants and their major components. CONCLUSION: regarding the lack of effective treatments for recruiting normal smell in many cases, treatments suggested by Avicenna worth entering pharmacologic experiments and clinical trials.

Association Between Breast Reconstruction Surgery and Quality of Life in Iranian Breast Cancer Patients.

Breast reconstruction (BR) surgery is not common for the treatment of breast cancer in low- and middle-income countries, including Iran. We evaluated the quality of life (QoL) in Iranian breast cancer patients who underwent BR at the Cancer Institute of Iran. We compared patients who had BR with breast cancer patients who had a radical mastectomy as the control group, matched for age, and time since surgery. We interviewed the cases and controls and collected data about QoL using EORTC-Q30 and EORTC-Q23 questionnaires. We also obtained personal and clinical data for the patients and controls. We compared 61 BR and 45 radical mastectomy patients. The BR patients had a higher level of education (73.8%) than the mastectomy patients (27.3%). In addition, the BR patients had a higher employment rate (58%) than the mastectomy patients (4.4%). QoL was significantly better among BR patients compared to the control group (P<0.05). In the multivariable analyses, the BR patients had significantly lower scores of pain, fatigue, and diarrhoea than the controls. Breast cancer patients who underwent BR surgery had a higher quality of life scores in some domains compared to the radical mastectomy. Socioeconomic factors and awareness of the patients about BR was crucial for choosing BR among Iranian patients.

Late-onset pompe disease in Iran: A clinical and genetic report.

INTRODUCTION: Pompe disease is characterized by absence or deficiency of acid α-glucosidase, and several causative mutations are known. In this study we report clinical and laboratory data in Iranian patients with late-onset Pompe disease (LOPD), focusing on population-specific mutations. METHODS: Clinical and laboratory data of 14 patients from 10 families with the diagnosis of LOPD were recorded. All had reduced enzyme activity on dried blood spot (DBS) analysis. Genetic investigation was performed to identify the underlying mutations. RESULTS: The age of onset ranged from <2 to 38 years. The clinical presentations were heterogeneous. Two siblings presented with foot drop. The most common mutation was c.(-32-13T>G). There were 4 novel mutations: c.(2040 + 2dup); c.(1650delG); c.(1837T>G); and c.(2596delG). CONCLUSION: This is a comprehensive report of LOPD in Iranian patients. Distinct phenotypic and genotypic features in this population are highlighted. Muscle Nerve 55: 835-840, 2017.

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.