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.
Melika Rafiemehr; Ali Asghar Okhovat; Kamyar Moradi; Gilda Kiani Mehr; Shahriar Nafissi; Farzad Fatehi
Abstract
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. ...
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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.
Ali Asghar Okhovat; Behnaz Ansari; Helia Hemasian; Bahram Haghi-Ashtiani; Soroor Advani; Bentolhoda Ziaadini; Siamak Abdi; Hajir Sikaroudi; Shahriar Nafissi; Farzad Fatehi
Abstract
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 ...
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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.
Kamyar Moradi; Shirin Jamal-Omidi; Maryam Masoudi; Sayna Bagheri; Shahriar Nafissi; Farzad Fatehi
Abstract
Background: Neuromuscular disorders affect physical and mental aspects of a patient and in other words alter the patients’ quality of life (QOL). In the present study, we investigated the validity and reliability of the Persian version of Individualized Neuromuscular QOL (INQOL) to provide a better ...
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Background: Neuromuscular disorders affect physical and mental aspects of a patient and in other words alter the patients’ quality of life (QOL). In the present study, we investigated the validity and reliability of the Persian version of Individualized Neuromuscular QOL (INQOL) to provide a better insight into patients’ QOL. Methods: Original version of the INQOL was translated backward and then forward. The resultant Persian version and a standard questionnaire, 36-Item Short Form Health Survey (SF-36), were then given to 83 participants with neuromuscular disorders. Internal consistency, known-group validity, concurrent validity, and test-retest reliability were assessed. Results: The scores of matched sections for QOL in the two questionnaires were favorably correlated (P < 0.05). Correlation between test and retest scores was also significant (P < 0.05). Moreover, the Cronbach’s alpha of 0.82 was representative of robust internal consistency between INQOL covering sections. Conclusion: The Persian version of the INQOL can be used in clinical and research practice to detect changes in QOL which are related to neuromuscular disorders, due to its favorably reliable and valid characteristics.
Davood Fathi; Shahriar Nafissi; Shahram Attarian; Christoph Neuwirth; Farzad Fatehi
Volume 18, Issue 3 , August 2019, , Pages 119-126
Abstract
Motor unit number index (MUNIX) is an electrophysiological technique to give an estimate of functioning motor neurons in a muscle. For any given neurophysiological technique for the use in clinical or research studies, reproducibility between different operators and in a single operator in different ...
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Motor unit number index (MUNIX) is an electrophysiological technique to give an estimate of functioning motor neurons in a muscle. For any given neurophysiological technique for the use in clinical or research studies, reproducibility between different operators and in a single operator in different times is one of the most important qualities, which must be evaluated and approved by different examiners and centers. After its introduction, testing the reproducibility of MUNIX was the aim of many studies to show this quality of the technique. In this review, we aimed to summarize all the studies, which have been performed up to now to approve MUNIX reproducibility in amyotrophic lateral sclerosis comparing healthy individuals.
Mohammad Mehdi Heidari; Mehri Khatami; Shahriar Nafissi; Faezeh Hesami-Zokai; Afshin Khorrami
Volume 14, Issue 4 , October 2015, , Pages 190-194
Abstract
Background: Non-dystrophic myotonias are a heterogeneous set of skeletal, muscular channelopathies, which have been associated with point mutations within sodium channel α-subunit (SCN4A) gene. Because exons 22 and 24 of SCN4A gene are recognized as hot spots for this disease, the purpose of the study ...
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Background: Non-dystrophic myotonias are a heterogeneous set of skeletal, muscular channelopathies, which have been associated with point mutations within sodium channel α-subunit (SCN4A) gene. Because exons 22 and 24 of SCN4A gene are recognized as hot spots for this disease, the purpose of the study is to identify mutation in exons 22 and 24 of SCN4A gene in Iranian non-dystrophic myotonias patients.Methods: In this study, 28 Iranian patients with non-dystrophic myotonia analyzed for the mutation scanning in exons 22 and 24 of SCN4A gene by polymerase chain reaction-single strand conformational polymorphism (PCR-SSCP) and sequencing. Results: We found 29073G>C substitution in SCN4A gene in one case and 31506A>G substitution in seven cases. The 29073G>C substitution causes a missense mutation G1306A, located in the conserved cytoplasmic loop connecting repeat III and IV of the SCN4A channel but, 31506A>G substitution do not alter amino acid in SCN4A protein.Conclusion: G1306A residue is located in functionally important protein region. In “hinged-lid model” for Na+ channel inactivation in which glycines1306 act as the hinge of the lid occluding the channel pore. Mutation in this region slowed fast inactivation. Therefore, it might be a pathogenic mutation. The causal relationship of this mutation with the disease is an object for further discussion.
Marzieh Khani; Afagh Alavi; Shahriar Nafissi; Elahe Elahi
Volume 14, Issue 3 , July 2015, , Pages 152-157
Abstract
Background: Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disorder in European populations. ALS can be sporadic ALS (SALS) or familial ALS (FALS). Among 20 known ALS genes, mutations in C9orf72 and superoxide dismutase 1 (SOD1) are the most common genetic causes of the disease. ...
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Background: Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disorder in European populations. ALS can be sporadic ALS (SALS) or familial ALS (FALS). Among 20 known ALS genes, mutations in C9orf72 and superoxide dismutase 1 (SOD1) are the most common genetic causes of the disease. Whereas C9orf72 mutations are more common in Western populations, the contribution of SOD1 to ALS in Iran is more than C9orf72. At present, a clear genotype/phenotype correlation for ALS has not been identified. We aimed to perform mutation screening of SOD1 in a newly identified Iranian FALS patient and to assess whether a genotype/phenotype correlation for the identified mutation exists.Methods: The five exons of SOD1 and flanking intronic sequences of a FALS proband were screened for mutations by direct sequencing. The clinical features of the proband were assessed by a neuromuscular specialist (SN). The phenotypic presentations were compared to previously reported patients with the same mutation.Results: Heterozygous c.260A > G mutation in SOD1 that causes Asn86Ser was identified in the proband. Age at onset was 34 years and site of the first presentation was in the lower extremities. Comparisons of clinical features of different ALS patients with the same mutation evidenced variable presentations.Conclusion: The c.260A > G mutation in SOD1 that causes Asn86Ser appears to cause ALS with variable clinical presentations.
Samira Yadegari; Shahriar Nafissi; Neda Kazemi
Volume 13, Issue 3 , September 2014, , Pages 138-143
Abstract
Background: Incidence and predominant subtype of Guillain-Barre syndrome (GBS) differs geographically. Electrophysiology has an important role in early diagnosis and prediction of prognosis. This study is conducted to determine the frequent subtype of GBS in a large group of patients ...
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Background: Incidence and predominant subtype of Guillain-Barre syndrome (GBS) differs geographically. Electrophysiology has an important role in early diagnosis and prediction of prognosis. This study is conducted to determine the frequent subtype of GBS in a large group of patients in Iran and compare nerve conduction studies in axonal and demyelinating forms of GBS.Methods: We retrospectively evaluated the medical records and electrodiagnostic study (EDS) of 121 GBS patients who were managed in our hospital during 11 years. After regarding the exclusion criteria, patients classified as three groups: acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN). The most frequent subtype and then electrophysiological characteristic based on the time of EDS and their cerebrospinal fluid (CSF) profile were assessed.Results: Among 70 patients :nally included in the study, 67% w re men. About 63%, 23%, and 14% had AIDP, AMAN, and AMSAN, respectively.AIDP patients represented a wider range of ages compared with other groups. Higher levels of CSF protein, abnormal late responses and sural sparing were more frequent in AIDP subtype. Five AMSAN patients also revealed sural sparing. Conduction block (CB) was observed in one AMAN patient. Prolonged F-wave latency was observed only in AIDP cases. CB and inexcitable sensory nerves were more frequent after 2 weeks, but reduced F-wave persistency was more prominent in the early phase.Conclusion: AIDP was the most frequent subtype. Although the electrophysiology and CSF are important diagnostic tools, classification should not be made based on a distinct finding.
Sama Bitarafan; Mohammad-Hossein Harirchian; Shahriar Nafissi; Mohammad-Ali Sahraian; Mansoureh Togha; Fereydoun Siassi; Ahmad Saedisomeolia; Elham Alipour; Nakisa Mohammadpour; Maryam Chamary; Niyaz Mohammadzadeh Honarvar; Ali-Akbar Saboor-Yaraghi
Volume 13, Issue 1 , March 2014, , Pages 28-32
Abstract
Background: The role of nutrition in the progression of multiple sclerosis (MS) and related complications such as fatigue has been reported by several studies. The aim of this study is the assessment of nutritional status and its relationship with fatigue in multiple ...
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Background: The role of nutrition in the progression of multiple sclerosis (MS) and related complications such as fatigue has been reported by several studies. The aim of this study is the assessment of nutritional status and its relationship with fatigue in multiple sclerosis patients.Methods: This is a cross-sectional study, in which 101 relapsing-remitting MS patients were enrolled.The fatigue status was determined using the validated Persian version of the Modified Fatigue Impact Scale (MFIS). Dietary intake was assessed using a 3-day food record questionnaire and compared to dietary reference intake (DRI) values. Association between variables was determined using Pearson Correlation Coefficient.Results: In the preset study, 25 men and 76 women (total = 101) were enrolled. Analysis of dietary intake showed that daily intake of vitamin D, folate, calcium, and magnesium were significantly lower than DRI in all of patients. In men, zinc intake was significantly lower than DRI; while, in women, iron was significantly below the DRI level. After adjusting for energy, MFIS and its physical subscale were highly correlated with intake of folate and magnesium.Conclusion: Our findings support that lower magnesium and folate diets are correlated with higher fatigue scores in MS patients.
Hosein Shamshiri; Mohammad Reza Eshraghian; Nastaran Ameli; Shahriar Nafissi
Volume 12, Issue 3 , September 2013, , Pages 102-105
Abstract
Background: As a disease of motor nervous system (motor neuron disease), amyotrophic lateral sclerosis (ALS) has a great impact on several aspects of quality of life (QoL). Generic questionnaires of QoL do not address all the especial features of ALS and therefore translation ...
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Background: As a disease of motor nervous system (motor neuron disease), amyotrophic lateral sclerosis (ALS) has a great impact on several aspects of quality of life (QoL). Generic questionnaires of QoL do not address all the especial features of ALS and therefore translation and validation of disease specific questionnaires such as Amyotrophic Lateral Sclerosis Assessment Questionnaire 40-item (ALSAQ-40) is necessary for assessment of patients with different languages. The aim of this study was to review the validation of the Persian version of the ALSAQ-40.Methods: Meticulously translated ALSAQ-40 was completed by 21 ALS patients. Internal reliability was evaluated using Cronbach’s alpha coefficient and item- total correlation was also used to evaluate the correlation of each question with total score.Validity was evaluated through comparison with Amyotrophic Lateral Sclerosis Functional Rating Scale-revised (ALSFRS-r)and the 36-Itemhort Form Health Survey (SF-36). Results: Cronbach’s alpha coe<cient was 0.91-0.96 for different scales of the ALSAQ-40. All the 40 questions of the questionnaire had correlation greater than 0.5. Correlation coefficient of all the related scales of the Persian version of ALSAQ-40, SF-36 and ALSFRS-r was greater than 0.59 with P value < 0.001.Conclusion: Measures of the Cronbach’s alpha coefficient and item-total correlation demonstrated reliability and consistency of the questionnaire, and correlation coefficients confirmed the validity of different items in the questionnaire. This study showed that the Persian version of the ALSAQ-40 is a reliable and valid questionnaire for the evaluation of QoL in ALS patients with Persian language.
Azin Ayatollahi; Mohammad Reza Mohajeri-Tehrani; Shahriar Nafissi
Volume 12, Issue 1 , March 2013, , Pages 19-22
Abstract
Background: Multiple sclerosis (MS) is a demyelinating disease which can cause many disabilities for the patient. Recent data suggests that MS patients have higher risk for osteoporosis. This study was performed to investigate if the osteoporosis prevalence is higher in MS patients and to determine the ...
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Background: Multiple sclerosis (MS) is a demyelinating disease which can cause many disabilities for the patient. Recent data suggests that MS patients have higher risk for osteoporosis. This study was performed to investigate if the osteoporosis prevalence is higher in MS patients and to determine the possible factors affecting bone mineral density (BMD).Methods:51 definite relapsing-remitting MS patients according to McDonald's criteria (45 females, 6 males aged between 20 and 50 years) participated in this study. The control group included 407 females aged from 20 to 49 years; they were healthy and had no history of the diseases affecting bone metabolism. Femoral and lumbar BMD were measured by Dual Energy X-ray Absorptiometry (DXA). The disability of MS patients was evaluated by Expanded Disability Status Scale (EDSS).Results:The patient's quality of life was evaluated by the validated Persian version of multiple sclerosis impact scale (MSIS-29).Patients' mean age was 36 ± 3.3 years and their mean disease duration was 8.7 ± 1.7 years. The mean EDSS score and the mean body mass index (BMI) of the patients were 3 ± 0.9 and 23.5 ± 2.3 kg/m(2), respectively. 29% of the patients had never been treated by ß-interferon and 6% of them had not received glucocorticoids (GCs) pulses since their MS had been diagnosed. 26% of the patients had a history of fracture.18% of our patients were osteoporotic and 43% of them were osteopenic. Femoral BMD was significantly lower among MS patients than age matched controls (P < 0.001), but lumbar BMD showed no difference. There was no correlation between administration of GCs pulses, interferon and BMD; however, we found a significant correlation between EDSS score, quality of life (QoL), disease duration and BMD of both site.Conclusion:As a result of this study, bone loss inevitably occurs in MS patients.The major factor of BMD loss is immobility. Osteoporosis should be managed as part of MS patients' treatment protocols.
Samira Yadegari; Askar Ghorbani; Mitra Ansari Dezfouli; Shahriar Nafissi
Volume 10, 3-4 , December 2011, , Pages 54-7
Abstract
Brown-Vialetto-Van Laere syndrome (BVVLS) is a rare neurological disorder. We report our finding about four patients clinically and electrophysiologically diagnosed as BVVLS and denoted their clinical features with comparison to previous reports. The first symptom was bilateral hearing loss and the onset ...
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Brown-Vialetto-Van Laere syndrome (BVVLS) is a rare neurological disorder. We report our finding about four patients clinically and electrophysiologically diagnosed as BVVLS and denoted their clinical features with comparison to previous reports. The first symptom was bilateral hearing loss and the onset of other cranial nerves involvement varied between 0-15 years. Our patients represented some rare features like upper motor neuron signs in one patient and two cases interestingly developed some clinical improvement. This is the first report of BVVLS from Iran. Our patients which represent Caucasian population had generally similar features like previously reported cases.
