scholarly journals P.075 Inflammatory Myositis associated with Myasthenia Gravis with and without thymic pathology: case series and literature review

Author(s):  
K Huang ◽  
M Mezei ◽  
K Shojania ◽  
N Amiri ◽  
N Dehghan ◽  
...  

Background: The association of myasthenia gravis (MG) and inflammatory myositis (IM) is rare and often only one of the diseases is diagnosed. Methods: In this study, we reviewed medical records of patients seen at NMDU from 2004 to 2017 who had diagnosis of concurrent MG and IM. The data is presented descriptively. -Results: We identified 7 patients with MG-IM overlap. Clinical features, laboratory and pathology data of the patients are summarized in Table 1. Conclusions: This is one of the largest case series with MG-IM overlap. It is very important to recognize such association and the different pattern of muscle involvement because therapies may be adjusted to treat both conditions. In patients with thymic pathology, conventional disease modifying agents, IVIG and glucocorticoid in addition to thymoma resection appear to be effective. In patients with refractory MG and myositis who were AChR negative, rituximab may be effective.

2021 ◽  
Author(s):  
Cleonisio Leite Rodrigues ◽  
Hermany Capistrano Freitas ◽  
Paulo Reges Oliveira Lima ◽  
Pedro Helder de Oliveira Junior ◽  
José Marcelino Aragão Fernandes ◽  
...  

Abstract Myasthenia Gravis (MG) is an autoimmune disorder of the neuromuscular junction that can be exacerbated by many viral infections, including COVID19. Management of MG exacerbations is challenging in this scenario. We report 8 cases of MG exacerbation or myasthenic crisis associated with COVID19 and discuss prognosis and treatment based on a literature review. Most patients were female (7/8), with an average age of 47.1 years. Treatment was immunoglobulin (IVIG) in 3 patients, plasma exchange (PLEX) in 2 patients, and adjustment of baseline drugs in 3. In-hospital mortality was 25% and 37.5% in 2-month follow-up. This is the largest case series of MG exacerbation or myasthenic crisis due to COVID19 to this date. Mortality was considerably higher than in myasthenic crisis of other etiologies. Previous treatment for MG or acute exacerbation treatment did not seem to interfere with prognosis, although sample size was too small to draw definitive conclusions. Further studies are needed to understand the safety and effectiveness of interventions in this setting, particularly of PLEX, IVIG, rituximab and tocilizumab.


2021 ◽  
Vol 15 (2) ◽  
pp. 91
Author(s):  
Desak Putu Gayatri Saraswati Seputra ◽  
I Gusti Ayu Sri Mahendra Dewi

Introduction: Thymoma is a neoplasm of the thymus gland which arises from the epithelial tissue. Thymoma is frequently related to various paraneoplastic syndromes, most commonly myasthenia gravis. Up to 50% of patients with thymoma are reported to develop myasthenia gravis, whereas 10–15% of patients with myasthenia gravis are found to have thymoma. Case Presentation: This case series reported 3 patients with myasthenia gravis which co-occurred with thymoma at Sanglah General Hospital from 2018 to 2019. The patients’ ages range from 33 to 61 years (mean age 46 ± 11 years) with a 2:1 male-to-female ratio. The diagnosis of myasthenia gravis was made by the presentation of typical myasthenia gravis appearances, the fluctuating intensity of ocular and bulbar muscle weakness (all three cases), and limbs and axial muscle weakness (one case); no respiratory muscle involvement was found. Physical and supporting examinations including electroneuromyography results were coherent with myasthenia gravis. The diagnosis of thymoma was made by highly suggestive appearance in Thorax Multi Sliced Computed Tomography (MSCT) scan examination and histopathologic findings. MSCT scan examination showed a well-circumscribed ovoid solid mass in the mediastinum (left anterosuperior, superior, and anterior to middle right mediastinum) sized from 1.8x1.5x2.9 cm to 8.1x9.9x9.7 cm, enhanced with contrast injection with areas of calcification and central necrotic (1 case) and no dissemination to regional lymph node and neighboring organs. All cases received acetylcholinesterase inhibitors and underwent extended thymectomy. Histopathological examination showed accordance with thymoma features of subtype A, AB, and B1.Conclusions: Clinicians should be aware of the thymoma and myasthenia gravis co-existence possibility to ensure early recognition and appropriate management.


2019 ◽  
Vol 2019 ◽  
pp. 1-4 ◽  
Author(s):  
Carina G. Colossi ◽  
Juliano Mondadori ◽  
Pedro K. M. Barreto ◽  
Felipe M. Valença ◽  
Rodrigo Duquia ◽  
...  

Background. Ophthalmic findings in mycosis fungoides (MF) can be highly variable. It seems that the prevalence of ophthalmic findings could be much more common than previously assumed. Objective. To present case series examined in the last 12 months, together with a literature review. Methods. Symptomatic patients with biopsy-proven mycosis fungoides were examined ophthalmologically in a 12-month period. The medical records of affected patients were reviewed. Results. Eight patients were examined. Of these, 75% were male, all were Caucasian, and average age was 58.2 years. Blepharitis (50.0%), thickened eyelids (37.5%), and flaking (25.0%) were the most prevalent findings. Conclusion. Incidence of MF affecting the eyes and surrounding structures may be greater than estimated. Early case management offers means to reduce difficulties experienced with later diagnosis. Regular monitoring by an ophthalmologist is justified, including that of asymptomatic cases.


Author(s):  
Mark A. Tarnopolsky ◽  
Erin Hatcher ◽  
Rachel Shupak

AbstractObjectives: Differentiating genetic myopathies from inflammatory myopathies can be challenging because of multiple overlapping clinical features. Examples are presented to highlight important clinical features that assist in the differentiation between the two. Methods: Clinical features including age at onset, history, pattern of weakness, serum creatine kinase activity, electromyography findings, and muscle biopsies are reported in six patients initially thought to have an inflammatory myopathy in whom the final diagnosis was a genetic myopathy. Results: All six patients met Bohan and Peter criteria for at least probable idiopathic polymyositis and were subsequently found to have a genetic myopathy (4 DYSF, RYR1, and GNE). The key distinguishing clinical were minimal to no response to immunosuppression and atypical involvement of distal muscles in the majority of cases. Conclusions: Patients diagnosed with inflammatory myopathies should be reevaluated for the possibility of a genetic myopathy if they fail to respond to a course of disease-modifying agents and/or there is atypical distal muscle involvement.


Author(s):  
Anurag Tiwari ◽  
Nishit Bhatnagar ◽  
Yugal Karkhur ◽  
Ammar Aslam ◽  
Amit Sharma ◽  
...  

<p class="abstract"><strong>Background:</strong> The purpose of this study was to review the cases of isolated tuberculous osteomyelitis of calcaneum in terms of clinical features, radiological features, treatment and outcome.</p><p class="abstract"><strong>Methods:</strong> Medical records of 9 patients (7 males and 2 females) aged 4 to 45 years (mean 21.33 years) who presented with isolated calcaneal tuberculous osteomyelitis were reviewed. Clinical features, laboratory tests result, findings of imaging modalities, and histopathological examination were noted. The location of involvement of tuberculosis and treatment offered were noted. Multifocal tuberculosis and articular involvement were excluded.<strong></strong></p><p class="abstract"><strong>Results:</strong> All patients presented with pain and swelling around the heel; three of them presented with a discharging sinus. Patients had involvement of calcaneal body (n=5), tuberosity (n=2), and anterior process (n=2). The erythrocyte sedimentation rate of all patients was elevated. All the patients were treated conservatively with immobilization and chemotherapy.</p><strong>Conclusions:</strong> Isolated calcaneal tuberculosis is rare and the prognosis with non-operative treatment is good.


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