Emad Alaei Tafti; Marjan Ghazisaeedi; Payam Sarraf
Volume 22, Issue 1 , January 2023, , Pages 35-43
Abstract
Background: Failure in early diagnosis of myasthenia gravis (MG) and the risks of taking certain medications and undergoing surgery and anesthesia can lead to severe respiratory disorders and death. However, there are therapeutic measures that significantly control the disease and improve individual’s ...
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Background: Failure in early diagnosis of myasthenia gravis (MG) and the risks of taking certain medications and undergoing surgery and anesthesia can lead to severe respiratory disorders and death. However, there are therapeutic measures that significantly control the disease and improve individual’s functionality.Methods: First, an expert panel was formed, and a needs assessment questionnaire was prepared for the information elements and the capabilities required for the application and provided to neurologists with a subspecialty fellowship in neuromuscular diseases. Then, based on the analyzed results, the application was designed and created in 2 versions (physician and patient), and in 2 languages (Persian and English). Eventually, a questionnaire for user interaction and satisfaction was provided to 5 relevant physicians to evaluate the application.Results: The results showed that neurologists considered all items of the needs assessment questionnaire to be 100% essential. The capabilities of the application included registering the medication name and dose, recording symptoms and complaints by the patient, completing standard questionnaires, online chat, medication reminder, sending alerts to the doctor when the patient is unwell, and providing a variety of reports. The usability evaluation showed that neurologists evaluated the application at a good level with the average score of 8.23 ± 0.47 (out of 9 points).Conclusion: In the long run, using this technology can reduce costs, improve patients’ quality of life (QOL) and health care, change health behaviors, and ultimately, improve individual’s health.
Bentolhoda Ziaadini; Narges Karimi; Akram Panahi; Ali Asghar Okhovat; Farzad Fatehi; Shahriar Nafissi
Abstract
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 ...
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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.
Shahriar Faghani; Ali Asghar Okhovat; Narges Karimi; Mana Moassefi; Seyed Farzad Maroufi; Shahriar Nafissi; Farzad Fatehi
Abstract
Background: The 8-item myasthenia gravis activity of daily living (MG-ADL) questionnaire is a valid and reliable instrument for evaluating myasthenia gravis (MG)-associated disability. This study aims to assess its validity and reliability in the Iranian population.Methods: A total number of 58 patients ...
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Background: The 8-item myasthenia gravis activity of daily living (MG-ADL) questionnaire is a valid and reliable instrument for evaluating myasthenia gravis (MG)-associated disability. This study aims to assess its validity and reliability in the Iranian population.Methods: A total number of 58 patients with MG were qualified for the examination. All 58 patients completed the Persian translation of 15-item Myasthenia Gravis Quality of Life (MG-QOL15) and MG-ADL questionnaires initially, and 30 filled out the MG-ADL questionnaire 2 to 4 weeks after the initial visit. Pearson correlation coefficient of questionnaires, internal consistency using Cronbach's alpha (α), and test-retest repeatability of the questionnaire were evaluated.Results: The Pearson correlation coefficient of Persian versions of MG-QOL and MG-ADL was 0.93 (P < 0.01). The Persian MG-ADL showed satisfactory internal consistency (Cronbach’s α = 0.89) and test-retest reliability (r = 0.99, P < 0.01).Conclusion: The Persian MG-ADL is a valid and reliable questionnaire for the determination of activities of daily life in Iranian patients with MG.
Zahra Vahabi; Ferdos Nazari; Farzad Fatehi; Valiolah Bayegi; Zahra Saffarian; Fatemeh Saffarian; Shahriar Nafissi
Abstract
Background: Myasthenia gravis (MG) affects the neuromuscular transmission, causing fluctuating muscle weakness and fatigue. This study is carried out with the aim to study the electrophysiologic findings of different subtypes of MG referred to our center in Tehran, Iran. Methods: All patients with MG ...
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Background: Myasthenia gravis (MG) affects the neuromuscular transmission, causing fluctuating muscle weakness and fatigue. This study is carried out with the aim to study the electrophysiologic findings of different subtypes of MG referred to our center in Tehran, Iran. Methods: All patients with MG presenting to neurology department of Shariati Hospital, Tehran University of Medical Sciences were enrolled. Clinically, patients with MG were categorized as ocular vs. generalized. The acetylcholine receptor (Ach-R) and muscle-specific receptor tyrosine kinase (anti-MuSK) antibodies were performed. Repetitive Nerve Stimulation (RNS) was performed using the standard method, with supramaximal stimulation of muscles at the 3 Hz frequency by surface electrode at rest. Abductor pollicis brevis (APB) (median nerve), anconeus (radial nerve), trapezius (accessory nerve), and nasalis (facial nerve) muscles were studied in all patients. Single fiber electromyography (SFEMG) was performed by standard method. Results: 196 seropositive patients with MG were included in the study. In electrophysiological studies, RNS was performed for 146 patients of Ach-R-Ab positive MG, with positive results in 110 patients. In addition, SFEMG was conducted for 8 patients with negative RNS, which resulted in 7 positive tests. Among 23 patients with anti-MuSK-positive MG, RNS was performed for 16 patients, with positive results in 11 patients. The 5 remaining patients with negative RNS test were studied by SFEMG, 4 of whom had positive results. APB compound muscle action potential (CMAP) decrementation significantly correlated with Ach-R-Ab positive MG (P < 0.03). Conclusion: This finding can support the hypothesis that the selection of muscles in electrodignostic study would be important. The electrodiagnostic studies are a good and non-invasive diagnostic tool for MG, and a combination of different distal, proximal, and facial muscles can increase the overall sensitivity of the test.
Masoud Etemadifar; Mahsa Akafzadeh-Savari; Mehri Salari; Amirhossein Akhavan Sigari; Sara Ebrahimi-Pelarti; Nahad Sedaghat; Koorosh Etemad
Abstract
Background: Coronavirus disease 2019 (COVID-19) is spreading rapidly and has affected millions of people worldwide. Comorbid diseases have complicated the course of infection and increased mortality. Myasthenia gravis (MG) affects the neuromuscular junctions (NMJs) and can compromise respiratory muscle ...
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Background: Coronavirus disease 2019 (COVID-19) is spreading rapidly and has affected millions of people worldwide. Comorbid diseases have complicated the course of infection and increased mortality. Myasthenia gravis (MG) affects the neuromuscular junctions (NMJs) and can compromise respiratory muscle action, leading to worse clinical outcomes in individuals infected with the COVID-19 theoretically. In this study, the aim is to assess the pattern of COVID-19 infection in patients with MG based on several factors. Methods: This was a prospective cohort study following 150 patients with MG over a six-month period. The patients were monitored for the development of signs and symptoms of the COVID-19 infection. Results: Comparison of the patients infected with COVID-19 with MG and those not infected was performed independently based on age, duration since MG diagnosis, status of thymectomy, and current clinical status of MG disease. Data analysis did not reveal increased susceptibility or increased severity of COVID-19 illness based the criteria assessed. Conclusion: COVID-19 related deaths and susceptibility were not related to age, thymectomy status, and disease duration in patients with MG.
Reza Boostani; Naghmeh Sadat Taheri
Kiana Amani; Payam Sarraf; Alireza Abkhoo; Abbas Tafakhori; Farzad Fatehi; Sama Bitarafan; Elmira Agah; Mamak Shariat
Abstract
Background: Myasthenia gravis (MG) is a chronic neuromuscular disease, which physically and mentally affects the patient’s life, with depression being one of the most important psychological complications in these patients. This study aims to investigate the prevalence of depression and its associated ...
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Background: Myasthenia gravis (MG) is a chronic neuromuscular disease, which physically and mentally affects the patient’s life, with depression being one of the most important psychological complications in these patients. This study aims to investigate the prevalence of depression and its associated factors in a group of Iranian patients with MG. Methods: This was a cross-sectional study in which consecutive patients diagnosed with MG who referred to two referral neuromuscular clinics affiliated to the Tehran University of Medical Sciences, Tehran, Iran, were evaluated for eligibility. Patients with a previously known psychiatric disorder and those with a family history of mental disorders were excluded. Eligible patients were interviewed and screened for depression through the administration of the Hamilton Depression Rating Scale (HDRS) and the Iranian version of Beck Depression Inventory-II (BDI-II). Results: A total of 62 patients participated in this study. The total prevalence of depression according to the HDRS and BDI-II scores was 64.5% and 53.2%, respectively. The mild depression was the most frequent level of depression based on the HDRS (33.9%) and BDI-II (22.6%) scores. None of the variables, including age, sex, duration of the disease, and dosages of prednisolone, pyridostigmine, and azathioprine, were correlated with the severity of depression. The number of academic years was the only variable associated with the lower HDRS score (P = 0.037). Conclusion: Based on the current findings, depression was common among Iranian patients with MG. The severity of depressive symptoms was unrelated to age, sex, marital status, duration of the disease, the daily dosage of medications, and thymectomy status. Further investigations are needed to reveal the exact burden of depression in patients with MG and address the importance of preventive interventions for improving the quality of life (QOL) in these patients.
Ali Asghar Okhovat; Farzad Fatehi
