The Epidemiologic, Clinical and Laboratory Findings of Patients with Myasthenia Gravis in Southern Iran (original) (raw)

Clinical Manifestations, Accompanying Diseases, Complications, and Thymus Pathologies in 102 Patients with Myasthenia Gravis

2006

Background: Myasthenia gravis (MG) is the most common autoimmune neuromuscular disease that may occur in any age. Regarding to the controversies about the epidemiology of MG and lack of sufficient data about MG status in Iran, the present study was performed on patients referred to Shafa hospital, in Kerman, between 1998-2003 in order to determine clinical manifestations, accompanying diseases, complications, and thymus pathologies in patients with MG. Methods: It was a descriptive study including 102 patients with MG. Initial data about sex, age of disease onset, disease severity according to Osserman classification, respiratory crisis, thymus pathology, and accompanying diseases were recorded. Chi-square and independent t-test were used for statistical analysis. Results: The study population included 69(68%) females and 33(32%) males. For patients aged >40 years, female to male (F/M) ratio was 1:1. Disease manifestations were commenced before 20 and 40 years of old in 15.8% and 76.2% of patients respectively. Follicular hyperplasia of thymus was reported in 61.5%, whereas in 23% the pathology was normal or atrophy and in 15.4% was thymoma. Ocular involvement was found in 20.6% of patients. Based on Osserman classification, severity of IIA, IIB, III and IV were reported in 43.1, 21.6, 10.8, and 3.9% of the subjects, respectively. Conclusion: F/M ratio, age of disease onset, pathology of thymus, disease severity, and accompanying diseases have shown slight differences with prior studies. Epidemiological studies in other parts of the country are suggested.

Clinical delineation of myasthenia gravis in the Kingdom of Bahrain

Neurosciences, 2022

Methods: This was a retrospective, observational cohort study carried out at 3 hospitals in Bahrain. MG was classified into ocular or generalized types. We subdivided MG into early-onset)EOMG, ≤ 49 Original Article years(or late-onset)LOMG, > 49 years(. Demographic and clinical data were recorded. The data was entered and analyzed using SPSS version 26.0. Results: 61.2% were females. The mean age at onset was 43.8±17.7 years in males and 43.1±15.7 years in females. 72.4% had EOMG. A pure ocular presentation was most common)51%(. Limb weakness was more prevalent in AChR-positive patients. The MuSK group had more severe presentation. 57.1% of patients were AChR-positive, 3.1% MuSK-positive, and 39.8% double-seronegative. Generalized disease onset was more likely with AChR. Abnormal CT chest was seen in 24/69)35%(including thymic hyperplasia, thymoma, and thymic atrophy. Pathology findings were thymic hyperplasia)55.0%(, thymoma)30%(, thymolipoma)10%(, and normal thymus)5%(. Treatment outcomes were favorable. Conclusion: The present study revealed that MG was more common in females, with similar age at onset between males and females. The majority of patients had EOMG with ocular disease and AChR positivity. The clinical outcomes were favorable. Following a standardized protocol for MG diagnosis and workup is recommended.

Myasthenia Gravis in Iranian Children

Iranian journal of child neurology, 2007

Objective This study was undertaken to evaluate the clinical spectrum of myasthenia gravis in children and determine factors that help the clinician in his/her diagnosis and management. Materials & Methods A retrospective review was performed on all pediatric patients suffering from myasthenia gravis (M.G) admitted in the department of pediatric neurology of the Mofid Hospital of the Shaheed Beheshti University, between 1994 and 2002. Results Of the thirty-two children with M.G. enrolled in our study, seven were suffering from the congenital type while the remaining (25 cases) had the juvenile M.G. Initial symptoms of congenital M.G were ptosis (7/7), limitation of eye movement (2/7) and mild generalized weakness (6/7). Although the Tensilon test was positive in 85% of congenital M.G cases, no myasthenia crisis or spontaneous remission was observed in any of them. In children with juvenile M.G, the age of presentation was 1.2 to 12.5 years, mean age 5.7+4.2 years (15 girls and 10 boys). The most common presenting symptoms in juvenile group were ptosis in 96% and generalized weakness in 76%. Eight of them (32%) had had at least one myasthenia crisis. EMG was diagnostic in 83% and one case the tensilon test was positive in 84%. One patient had hyperthyroidism and had already been diagnosed with hypothyroidism; two of them were epileptics. Eight patients underwent thymectomy microscopically; in specimens examined, five (62%) showed thymic follicular hyperplasia while in remaining three results were normal. One patient (12.5%) recovered completely after thymectomy with no need for medication during the follow up. Four patients (50%) showed relative improvement and in three cases (37%) improvement was negligible. Conclusion The results showed a female to male ratio of 1.5/1 which was correlated to adult M.G. The most common presenting symptoms consisted of ophtalmoplegia, with bilateral ptosis being the most significant. Although this study revealed that thymectomy lacks any remarkable prognostic influence, all patients had thymectomy after two years of disease onset. Some reports have indicated positive results if surgery was performed within two years of onset of disease.

Serological and Clinical Features of Patients with Myasthenia Gravis in North Indian Population

International Journal of Neuroscience, 2010

Myasthenia gravis (MG) is a disorder of neuromuscular junction associated with presence of antibodies against nicotinic acetylcholine receptors (nAChRs). Here, we compared the clinical and serological profile of seropositive myasthenia gravis (SPMG) and seronegative myasthenia gravis (SNMG) patients. Anti-AChR antibody was measured using radio receptor immunoassay and correlated with clinical phenotype in 250 MG patients over 2004 and 2006. Out of 250 MG patients, 161 (64.4%) were males (male:female = 1.8:1). SNMG patients formed 40% (n = 101) of our MG patients which is much higher as compared to Caucasian and Oriental population (15%-20%). The median age of disease onset in SPMG was significantly higher than SNMG patients (43 years; range 8-74 vs. 35 years; range 4-72, p = .022). A bimodal peak of age of disease onset in both genders was observed (first peak in second-third decades and second one in fifth-sixth decades). Among the MG patients with late-onset of disease, male were significantly higher compared to Caucasian and Oriental MG population (p = .047). MG patients with thymoma were significantly older and consisted of higher percent of males. Bulbar symptoms and severe grade (IIB+ III+ IV) at disease onset were more frequent in SPMG than SNMG patients.

Myasthenia Gravis and Associated Diseases

Open Access Macedonian Journal of Medical Sciences, 2018

BACKGROUND: Myasthenia gravis (MG) is an autoimmune disease caused by the action of specific antibodies to the postsynaptic membrane of the neuromuscular junction, leading to impaired neuromuscular transmission. Patients with MG have an increased incidence of other autoimmune diseases.AIM: to determine the presence of other associated diseases in patients with MG.METHOD: A group of 127 patients with MG followed in 10 years period, in which the presence of other associated diseases has been analysed.RESULTS: The sex ratio is in favour of the female sex, the average age of the initial manifestation of the disease is less than 50 years, 65.4% of the patients with MG have another disease. 15.0% patients have associated another autoimmune disease. Thyroid disease is the most common associated with MG, rarely rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and other autoimmune diseases. Other diseases include hypertension, heart disease, diabetes, respiratory diseases, dysli...

Double Sero-Negative Myasthenia Gravis Associated with Thymic Hyperplasia and Autoimmune Thyroiditis in a Young Patient

Romanian Journal of Neurology, 2017

Myasthenia gravis is an autoimmune disorder affecting the postsynaptic neuromuscular junction. Clinically it evolves with fluctuating muscle weakness. In 13-22% of the reported cases, another autoimmune disorder is associated. We report the case of a 21 years old woman presenting at 4 months after delivrence, global muscle weakness, dysphonia, dysartria and dysphagia. Biological tests reveal: AchR antibodies and anti-MUSK antibodies – negative, TSH 4,68 μIU/ml (normal range: 0,4-4 μIU/ml), FT4 0,785 ng/dl (normal range: 0,89-1,76 ng/dl), anti thyroglobulin antibodies 183,01 IU/mL (normal range: <4,11 IU/mL), Chest CT – thymic hyperplasia and EMG – decremental response at repetitive stimulation. It is established the diagnosis: myasthenia gravis Osserman stage IIb. After treatment with acetylcholine esterase inhibitors and corticosteroids the patient`s symptomathology has improved.

Clinical and epidemiological aspects

2016

A retrospective chart review was performed on patients diagnosed as having myasthenia gravis in Ceará State, Brazil and who were followed from October 1981 to June 2009. Clinical and epidemiologic aspects were evaluated. In this work, 122 patients were studied, of whom 85 (69.7%) were females and 37 (30.3%) were males. The disease duration ranged from five months to 50 years (8.9±8.1 years). Age at the first symptoms varied from 0 to 74 years (31.9±14.4 years). The first main symptoms and signs were ptosis, diplopia and limb weakness. Generalized myasthenia was the most common clinical presentation, but 5.1 % (n=6) persisted as ocular myasthenia. Thymectomy was performed in 42.6 % (n=52) of myasthenic patients. A thymoma was present in 10 patients. Serum acetylcholine receptor (AChR) antibodies were present in 80 % (n=20) of specimens tested. The data presented are similar to those of studies performed in other countries.

Myasthenia Gravis and Autoimunne Thyroid Disease Association

Haydarpasa Numune Training and Research Hospital Medical Journal

Introduction: Autoimmune thyroid disease (AITD) frequently accompanies myasthenia gravis (MG). The aim of the present study was to evaluate the clinical and serological features of MG associated with AITD. Methods: Thirty patients diagnosed with MG between 2012 and 2016 were followed. All patients' clinical and demographic features were analyzed, and acetylcholine receptor antibody (AChRAb) and antimicrosomal antibody were evaluated. There were 30 (21 female and nine male) patients suffering from MG. Age range was 13-78 years. Age range at disease onset was 11-75 years. Duration of disease range was 1-38 years. Results: Of the 30 patients, 17 (56.7%) were positive for AChRAb, and 7 (23%) were positive for antimicrosomal antibody. Among 17 AChRAb (+) patients, both diagnoses of generalized (14/23 (60.9%)) and ocular (3/7 (42.9%)) myasthenia were present. Of the 17 AChRAb (+) patients, 6 were also positive for antimicrosomal antibody. All seven antimicrosomal antibody (+) patients had generalized myasthenia. Discussion and Conclusion: It is really important to think coexisting MG in patients with autoimmune disorders and neuromuscular weakness. The presence of AChRAb in patients with MG is associated with a frequent risk for other autoimmune diseases.

Clinical and serological study of myasthenia gravis using both radioimmunoprecipitation and cell-based assays in a South Asian population

2014

Background: Ocular and childhood myasthenia gravis (MG) cases seem relatively more common in Oriental than in Caucasian populations, but there have been no comprehensive serological studies on patients from mainland China. Methods: 391 unselected patients with MG attending Tongji Hospital in WuHan (the largest hospital in the province of HuBei, China) were studied during a 15-month period; most had already received treatment for their condition. Results: The male to female ratio was 0.8. 50% of the patients were children (,15 years), and age at onset showed a single peak at between 5 and 10 years of age. 64% of the children and 66% of the adults were positive for acetylcholine receptor (AChR) antibodies but the antibody titres were lower than in similar Caucasian studies, although this was partly due to the high incidence of ocular MG. Of the 43 patients with generalised MG without AChR antibodies, only 1 had muscle-specific kinase antibodies (2.5%) and 2 had voltage-gated calcium channel antibodies indicating probable Lambert-Eaton myasthenic syndrome. 75% of the children, compared with only 28% of the adults, had ocular MG. Thymoma was evident by MRI in 1.5% of children and in 20% of adults. Despite most patients having received prednisone, very few had obtained full clinical remission. Conclusion: This study emphasises the frequency of early childhood onset with ocular symptoms and shows that many of these patients have AChR antibodies. By contrast, patients presenting in later age seem to be very uncommon in comparison with recent studies in Caucasian populations.