The co-existence of myasthenia gravis in patients with myositis: A case series

ObjectiveMyositis and myasthenia gravis (MG) are both autoimmune disorders presenting with muscle weakness. Rarely, they occur simultaneously in the same patient. Since the management of myasthenia gravis differs from that of myositis, it is important to recognize when patients have both diseases. We reviewed the cases of 6 patients with both myositis and MG to identify clinical features that suggest the possibility of co-existing MG in myositis patients.MethodsWe identified 6 patients with dermatomyositis or polymyositis and MG. We reviewed their medical records to assess their clinical presentations, laboratory findings, and electrophysiological features.ResultsAll 6 patients had definite dermatomyositis or polymyositis by the criteria of Bohan and Peter as well as electrophysiologic and/or serologic confirmation of MG. Among overlap patients, 5/6 (83%) had bulbar weakness, 2/6 (33%) had ptosis, and 1/6 (17%) had diplopia. Fatigable weakness was noted by 5/6 (83%) patients. Treatment with pyridostigmine improved symptoms in 5/6 (83%) patients. High-dose steroids were associated with worsening weakness in 2/6 (33%) patients.ConclusionsProminent bulbar symptoms, ptosis, diplopia, and fatigable weakness should suggest the possibility of MG in patients with myositis. A suspicion of MG may be confirmed through appropriate electrophysiologic and laboratory testing. In those with myositis–MG overlap, high-dose steroids may exacerbate symptoms and pyridostigmine may play an important therapeutic role.     

The co-existence of myasthenia gravis in patients with myositis: A case series

Objective

Myositis and myasthenia gravis (MG) are both autoimmune disorders presenting with muscle weakness. Rarely, they occur simultaneously in the same patient. Since the management of myasthenia gravis differs from that of myositis, it is important to recognize when patients have both diseases. We reviewed the cases of 6 patients with both myositis and MG to identify clinical features that suggest the possibility of co-existing MG in myositis patients.

Methods

We identified 6 patients with dermatomyositis or polymyositis and MG. We reviewed their medical records to assess their clinical presentations, laboratory findings, and electrophysiological features.

Results

All 6 patients had definite dermatomyositis or polymyositis by the criteria of Bohan and Peter as well as electrophysiologic and/or serologic confirmation of MG. Among overlap patients, 5/6 (83%) had bulbar weakness, 2/6 (33%) had ptosis, and 1/6 (17%) had diplopia. Fatigable weakness was noted by 5/6 (83%) patients. Treatment with pyridostigmine improved symptoms in 5/6 (83%) patients. High-dose steroids were associated with worsening weakness in 2/6 (33%) patients.

Conclusions

Prominent bulbar symptoms, ptosis, diplopia, and fatigable weakness should suggest the possibility of MG in patients with myositis. A suspicion of MG may be confirmed through appropriate electrophysiologic and laboratory testing. In those with myositis–MG overlap, high-dose steroids may exacerbate symptoms and pyridostigmine may play an important therapeutic role.     

重症肌无力胸腺切除术后发生肌无力危象的危险因素分析

目的:回顾性分析研究重症肌无力患者胸腺切除术后发生肌无力危象的危险因素。 方法:对本医院1970年-2011年实施的胸腺切除术的306例MG患者进行回顾分析，利用Logistic回归从多种相关因素（例如年龄、分型、术前术后处理、手术方式、预后等）中筛选出危险因素。 结果:术后发生肌无力危象与患者发病年龄、手术时间、病程、术前分型、术前是否累及吞咽肌、术前是否发生危象、是否确定为胸腺瘤相关，并且关系着患者的预后。 结论:术前吞咽肌受累、术前发生危象、确诊胸腺瘤是预测胸腺切除术后肌无力危象发生的独立危险因素。     

Coexistence of myasthenia gravis and amyotrophic lateral sclerosis in a Bosnian male: an unusual clinical presentation

PurposeMyasthenia gravis (MG) and amyotrophic lateral sclerosis (ALS) are two different diseases. The coexistence of both of them is extremely rare and represents a diagnostic challenge which requires thoughtful interpretation of clinical characteristics.Case reportWe present the case of a 46-year-old Bosnian male who developed ALS five months after MG. Diagnosis of MG was based on elevated titers of anti-AchR antibodies, positive edrophonium test, and decremental responses on a repetitive nerve stimulation test while the diagnosis of ALS was based on clinical and neurophysiological findings: upper motor neuron signs in the lumbar region, lower motor neuron signs in the bulbar and cervical regions, generalized fasciculations and muscle atrophy and progressive asymetric muscle weakness together with active and chronic denervation in the cervical and lumbosacral region determined by electromyoneurography.ConclusionsThe coexistence of MG and ALS is rare and request an adequate interpretation of clinical symptoms. The relationship between these two diseases in as interesting phenomen to present.Key words: myasthenia gravis, amyotrophic lateral sclerosis, coexistence     

Myasthenia gravis: MuSK MG,late-onset MG and ocular MG

Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction which affects all striated muscles, resulting in fluctuating weakness. Approaching MG as a disease with subgroups having different clinical, serological and genetic features is crucial in predicting the progression and planning treatment. Three relatively less frequently seen subtypes of MG are the subject of this review: MG with anti-MuSK antibodies (MuSK MG), non-thymomatous late-onset MG (LOMG), and ocular MG (OMG). In addition to reviewing the literature, mainly from a clinical point of view, our experience in each of the subgroups, based on close to 600 patients seen over a 10 year period, is related. MuSK MG is a severe disease with predominant bulbar involvement. It is more common in women and in early-onset patients. With the use of high dose corticosteroids, azathioprine and more recently rituximab, outcome is favorable, though the patients usually require higher maintenance doses of immunosuppressives. LOMG with onset ≥ 50 years of age is more common in men and ocular onset is common. Frequency of anti-AChR and anti-titin antibodies are high. Although it can be severe in some patients, response to treatment is usually very good. OMG is reported to be more frequent in men in whom the disease has a later onset. Anti-AChR antibodies are present in about half of the patients. Generalization is less likely when symptoms remain confined to ocular muscles for 2 years. Low dose corticosteroids are usually sufficient. Thyroid disease is the most common autoimmune disease accompanying all three subgroups.Key words: Myasthenia gravis, MuSK MG, Late-onset MG, Ocular MG     

Severe respiratory failure and torsades de pointes induced by disopyramide in a patient with myasthenia gravis

Class 1a anti-arrhythmic drugs are often used for the treatment of atrial fibrillation (AF), but it is not well known that myasthenia gravis (MG)-like symptoms can be generated by their anti-cholinergic effects. We had a patient with MG who developed symptomatic MG aggravation after AF treatment with disopyramide. Symptomatic MG aggravation was followed by Takotsubo-shaped cardiomyopathy, QT prolongation, and torsades de pointes. We suggest that the anti-cholinergic effects of disopyramide can induce MG crisis and should therefore be carefully considered when disopyramide is used to treat AF in patients with MG.     

The spread of Mycoplasma genitalium among men who have sex with men

We report two instances of urethral-to-rectal transmission of Mycoplasma genitalium (MG) in men who have sex with men (MSM) couples. Such clear epidemiological correlation has to our knowledge not been published before. The urethral infections led to clinical symptoms, but the rectal infections did not. The rectum might serve as a reservoir for MG in MSM, but there is also some evidence from the literature that MG can cause proctitis. Our finding raises important questions about the role of MG as a pathogen among MSM. Any correlation with HIV transmission risk is currently unknown and needs further research.     

Changes in Tongue Pressure, Pulmonary Function, and Salivary Flow in Patients with Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease involving nerve cells that control voluntary muscle movement (Rowland LP, Shneider NA, N Engl J Med 344(22):1688–1700, 2001). The aim of this study were to determine the pattern of neurodegenerative change in (1) isometric tongue strength (ITS) and spontaneous saliva swallow (SSS) pressure, (2) saliva weight, and (3) forced vital capacity (FVC) in patients with ALS who present with primary spinal versus primary bulbar symptoms. Twenty-three consecutive patients (age = 48–80 years, mean = 59.5 years) were enrolled. Data were collected over three visits (12-week interval) for each group: 9 patients with bulbar symptoms and 14 with spinal symptoms. A significant difference was noted in SSS and ITS in the group with bulbar symptoms from Trial 1 to II and from Trial II to III. SSS and ITS showed a significant difference when comparing Trial I to III but not when comparing Trial I to II for the spinal symptom group, indicating that this group experienced a slower decline in SSS. Saliva production did not show a significant change in the bulbar symptom group but did in the spinal group. FVC was significantly different when comparing Trial I to III and Trial II to III for both groups. FVC, SSS, and ITS may be complimentary measures used as a gauge of an ALS patient’s ability to efficiently take oral nutrition and to support required alterations in diet consistency.     

Concurrent inflammatory myopathy and myasthenia gravis with or without thymic pathology: A case series and literature review

Objectives

The association of myasthenia gravis (MG) and inflammatory myositis (IM) is rare and typically only one of the diseases is present. The management of the 2 diseases differs, therefore it is important to recognize the concomitant presentation. Here, we report a case series of 7 patients with co-existing MG and IM with review of the literature.

Dropped head sign as the only symptom of myasthenia gravis

Neck extensor muscle weakness and the dropped head sign are associated with various neuromuscular disorders. However, these symptoms are comparatively rare in myasthenia gravis (MG). We report a MG case that presented with dropped head sign as the main symptom. A 55-year-old man developed subacute weakness of the neck extensor muscle and presented with dropped head. We established a diagnosis of MG based on the results of an edrophonium test and a voluntary single fiber electromyogram (vSFEMG), and a high serum antiacetylcholine receptor antibody level. This patient was treated with pyridostigmine and his neurological symptoms improved. There are reported cases of dropped head sign as the first symptom of MG, however, in those cases, other muscles showed weakness during the first few months after onset. In the present case, throughout the clinical course no other symptoms outside of dropped head sign were seen.     

Retrospective analyses of clinical features and therapeutic outcomes in thymectomized patients with myasthenia gravis at Shinshu University

OBJECTIVES: Thymectomy has become recognized as an integral element in the treatment of patients with myasthenia gravis (MG). Although the incidence of elderly-onset MG has recently been increasing, there is little data demonstrating the efficacy and complications of thymectomy in this population. To clarify this point, we divided the thymectomized patients with MG into young and elderly groups, and retrospectively examined their clinical features and therapeutic outcomes. PATIENTS AND METHODS: We reviewed 57 MG patients who had been admitted to our hospital between 1988 and 2002. The patients were classified into young (younger than 60) and elderly (60 or older) groups according to the age of onset, and the therapeutic outcomes of thymectomy were evaluated using myasthenic severity scales and the duration from operation to discharge. RESULTS: Myasthenic severity scales significantly improved after thymectomy in the elderly group both with (p<0.005) and without thymoma (p<0.05) compared with before. With regard to the duration from thymectomy to discharge, no significant difference could be found between the young and elderly groups, irrespective of associated thymoma. There were no serious complications during and after thymectomy in either the young or the elderly group. CONCLUSIONS: Despite various possible complications due to aging, thymectomy should be actively considered also in the treatment of elderly MG patients because it can reliably and safely improve myasthenic symptoms in combination with immunosuppressive agents, including corticosteroid.     

Comparative Study of Clinical Effects Between Plasma Adsorption and Double Filtration Plasmapheresis in Patients with Myasthenia Gravis

Abstract: Plasma exchange (PE) has been one of the most powerful treatments for patients with myasthenia gravis (MG) since Pinching et al. reported its clinical usefulness in 1976, despite the need for supplemental human plasma. However, new apheresis techniques, e.g., plasma adsorption (PA) and double filtration plasmapheresis (DFPP), which do not need human plasma, were developed and have been introduced for clinical use in MG. We compared the effects of these plasma purification therapies in patients with MG and found that DFPP improved such subjective symptoms as chest compression and general fatigue better than PA while both of them could decrease the serum level of acetylcholine receptor (AChR) antibodies and relieve objective muscle weakness to a similar degree. It may be that DFPP can remove some circulating pathogenic factors other than AChR antibodies more efficiently than PA.—     

Association of Smoking and Generalized Manifestations of Myasthenia Gravis

Objective Smoking is a known risk factor for the development and progression of autoimmune diseases. Previous studies in ocular myasthenia gravis (MG) patients showed that smoking is associated with the severity of symptoms and progression to generalized MG. However, whether smoking affects MG symptoms in patients with a broader clinical spectrum of presentations is unknown. Therefore, in this study, the associations of smoking with the clinical characteristics of MG were analyzed in a cohort of patients including those with generalized, seronegative, and thymoma-associated MG. Methods The smoking history was investigated in a cross-sectional study of 187 patients with MG followed in a referral hospital for neurology. The association of smoking with MG-activities of daily living score at survey, the presence of generalized manifestations, and the age of onset was assessed using multiple regression models. Results Neither current nor prior smoking habit was associated with the MG-activities of daily living score at survey. However, smoking exposure after MG onset was significantly associated with the presence of generalized manifestations during the disease course (odds ratio, 3.57; 95% confidence interval, 1.04, 12.3). The smoking history before or at onset of MG was not associated with the age of onset. Conclusion Smoking exposure after the onset is associated with generalized manifestations of MG in our cohort of patients with a broad clinical spectrum of presentations.     

Resolution of autoimmune oophoritis after thymectomy in a myasthenia gravis patient

Myasthenia gravis (MG) is an autoimmune disorder characterized by autoantibodies against acetylcholine receptors. MG is generally an isolated disorder but may occur concomitantly with other autoimmune diseases. We describe an eighteen-year-old girl with MG who was admitted to our clinic with secondary amenorrhea and diagnosed as autoimmune oophoritis. Since her myasthenic symptoms did not resolve with anticholinesterase therapy, thymectomy was performed. After thymectomy, her menses have been regular without any hormonal replacement therapy. To our knowledge, this is the first report on a patient with autoimmune ovarian insufficiency and MG in whom premature ovarian insufficiency resolved after thymectomy, without hormonal therapy.     

D-penicillamine induced myasthenia gravis in a patient with rheumatoid arthritis

We have encountered a 53 year-old female with myasthenia gravis (MG) possibly induced by the treatment with D-penicillamine (D-pc). She has had a long-standing history of rheumatoid arthritis (RA) prior to the onset of MG. The diagnosis for MG was made based on the clinical findings and tests including antiacetylcholine receptor antibody and EMG. Although the drug had been efficacious for RA, it was discontinued after the development of MG. The symptoms and signs for MG were immediately disappeared, whereas clinical manifestations for RA was relapsed shortly after. Since D-Pc has been frequently used for the treatment of RA, an awareness of this side effect appears to be of particular importance for physicians in clinical practice who deal with RA. Furthermore, it may also provide some important immunological insights into the study of pathogenesis of MG.     

Factors influencing improvement and remission rates after thymectomy for myasthenia gravis.

BACKGROUND: Thymectomy has become an accepted option in the treatment of myasthenia gravis (MG). However, the optimal selection of patients for surgery remains controversial. OBJECTIVE: The objective of the study is to define factors influencing improvement and remission rates after thymectomy for MG. METHODS: We retrospectively reviewed our experience in the surgical management of patients with MG over the last 20 years, and analyzed if patient's age, sex, preoperative Osserman Stage, thymic histology, duration of symptoms, or radiological findings influenced outcome. RESULTS: There were 25 women and 10 men. Most of them presented with Osserman stage IIA (n = 12) and stage IIB (n = 17). Hyperplasia of the thymus was observed in 15 patients (43%), thymoma in 12 (34%), and atrophic or normal thymus in 8 (23%). Computed tomography (CT) had a positive predictive value of 91% in detecting thymoma and of 80% in detecting thymic hyperplasia. Over a mean follow-up of 96 months, MG improved markedly in 26 patients. Postoperative improvement was significantly greater in patients with advanced MG (stage IIB, III and IV), whereas a higher rate of remission occurred in patients with mild MG (stage I and IIA), and in those with thymic hyperplasia. No relation was observed between outcome and age, sex, duration of symptoms and abnormal CT findings. CONCLUSIONS: The beneficial effects of thymectomy are characterized by postoperative clinical improvement in most patients with moderate to severe preoperative myasthenic involvement and by a higher remission rate in patients with thymic hyperplasia and/or mild myasthenic symptoms.     

Clinical significance of autoimmune thyroid disease in myasthenia gravis.

We investigated thyroid function and autoimmunity in 74 (61% females) consecutive patients with MG. 30 of these patients were tested twice: the time between the investigations ranging from 1 to 4 years. MG was diagnosed on the basis of typical clinical symptoms, a positive tensilone-test, and/or detectable acetylcholine antibodies and/or repetetive stimulation tests. Eye involvement was present in 86%, concurrent thymomas in 56%. The following parameters were measured in the serum of these patients by commercially available kits: free and total T4, TSH, TSH-Receptor-Antibodies ("TRAB"), antibodies against thyroglobulin (Tg-Ab), against thyroid microsomes (M-Ab) and against acetyl-choline-receptors . An age matched group of 50 patients (54% females) with no known thyroid disease from a cardiological ward and from the neurological outpatient department served as control. There was only 1 MG patient with overt thyroid dysfunction (iodine induced thyrotoxicosis in a patient with autonomous adenoma and no circulating thyroid autoantibodies detected at the second investigation). There were, moreover, 1 euthyroid MG-patient on L-thyroxine therapy with a history of Hashimoto's disease and positive thyroid autoantibodies and 1 other MG-patient with mildly elevated TSH without elevated thyroid antibodies, who has had subtotal thyroidectomy without substitution therapy years before for unknown reasons. Tg-Ab were positive (>360 IU/ml) in 5%, M-Ab were positive (>154 IU/ml) in 15% of the MG patients. The control group had 4% Tg-Ab and 6% M-Ab. TRAB levels were normal in all patients and controls. The relative increase in M-Ab frequency was not statistically significant (x-square test). We conclude from our results, that autoimmune thyroid disease may be associated with MG but that the occurrence of thyroid dysfunction induced by autoimmunity is a very rare phenomenon in MG.     

重症肌无力伴骨骼肌以外受损的临床特征分析

目的 对伴心脏、肝脏损害,瞳孔不等,听力障碍,手足血管舒缩功能及皮肤营养障碍和阳痿等少见症状的重症肌无力(MG)患者的特点进行分析,提出诊断和鉴别诊断的方法。方法 (1)给予MG患者胆碱酯酶抑制剂(ChEI)及免疫治疗,再复查病情和随访;(2)伴瞳孔不等和听力障碍患者肌注新斯的明1mg,30～120min后观察症状变化,后者同时检查脑干听觉诱发电位2次。结果 55例MG患者临床症状随典型的骨骼肌病态疲劳的症状改善而好转,一般不需要特殊治疗。结论 ChEI治疗MG患者合并上述少见症状均有效,是与其他疾病鉴别的重要依据,也证实了MG是一种广泛的自身免疫性疾病。     

Relation between ST and QRS vector changes and myoglobin release in acute myocardial infarction

We analysed serum time activity curves for myoglobin (MG) and changes in the ST and QRS vectors for 18 consecutive patients with acute myocardial infarction admitted within 4 h of the onset of pain. The MG release was completed 16 +/- 7 (7 to 36) h after onset of symptoms, and the QRS vector changes were completed after 14 +/- 5 (4 to 23) h. the ST vector decline ceased after 11 +/- 5 h. The temporal correlation between completion of: a) ST vectors and MG release was r = 0.78 (P less than 0.001); b) QRS vectors and MG release was r = 0.85 (P less than 0.001). Stepwise release of MG and changes in ST and QRS vectors were often related. Seventeen additional ST-peaks were followed by further MG-release in 13 instances and for 10 additional changes of the QRS vector eight were associated with further MG-release. We conclude that VCG changes and MG-release show a close temporal relationship. Additional events are often simultaneously reflected by these independent markers of myocardial ischaemia and necrosis.     

Myasthenia gravis--a rare presentation with tongue atrophy and fasciculation

We report the case of an unusual presentation of myasthenia gravis with tongue atrophy and fasciculation. Myasthenia gravis is an autoimmune condition associated with weakness and fatigability of voluntary muscles. In >50%, the initial symptoms and signs are related to extraocular muscle weakness, such as diplopia or ptosis [Tsung K, Seggev JS. An unusual cause of dysphagia. West J Med 1995; 163: 159-60]. Rarely, it is known to affect bulbar muscles and can lead to dysphagia and respiratory compromise.