scholarly journals Clinical Presentation of Myasthenia Gravis

Thymus ◽  
2020 ◽  
Author(s):  
Jianu Catalin ◽  
Jianu Silviana ◽  
Barsan Claudia

Author(s):  
Jan B.M. Kuks ◽  
Hans J.G. H. Oosterhuis


2021 ◽  
Vol 2 (4) ◽  
Author(s):  
Olivia Watson ◽  
Michelle Jack ◽  
Helen Young

Myasthenia gravis and Graves’ disease are known to co-exist in adults, yet there have only been a small number of paediatric cases reported. We report a 5 year old female who was diagnosed with ocular myasthenia gravis after presenting with unilateral ptosis and subsequently found also to have Graves’ disease. She was treated successfully with pyridostigmine, corticosteroids and carbimazole without symptom recurrence or progression to generalised myasthenia gravis. The aetiology of the coexistence is not fully understood, nor is the relationship between the two disorders’ presentation and treatment. We discuss the variation in clinical presentation of myasthenia gravis between populations and when associated with autoimmune thyroid disease, potential HLA-related genetic susceptibility and the varying approaches to treatment of the co-existent disorders.



2018 ◽  
Vol 69 (8) ◽  
pp. 2251-2253
Author(s):  
Paul Salahoru ◽  
Cristina Mihaela Ghiciuc ◽  
Cristina Grigorescu ◽  
Marius Valeriu Hinganu ◽  
Catalina Elena Lupusoru

Myasthenia gravis is an autoimmune disorder which presents a series of clinical manifestations which are generally motor. The hallmark of the disorder is muscle weakness, which typically worsens during physical exercise and improves upon rest. Approximatively 80% of the patients presenting this affection test positive for Acetylcholine antibody anti-receptor (AChR-ab).Treatment of patients with myasthenia gravis consists of:administration of oral medica-tion, immunomodulating treatment by removal of AChR-ab through plasmapheresis or surgical intervention (thymectomy).This article presents a retrospective study in which the authors attempted to identify factors which can influence the effect of thymectomy on AChR-ab titration and on the clinical presentation of the patients suffering with myasthenia gravis.



2018 ◽  
Vol 50 (02) ◽  
pp. 116-121
Author(s):  
Hanene Benrhouma ◽  
Hedia Klaa ◽  
Rania Ben Aoun ◽  
Aida Rouissi ◽  
Melika Ben Ahmed ◽  
...  

AbstractMyasthenia gravis (MG) with antibodies to the muscle-specific tyrosine kinase (MuSK-MG) receptor is a rare entity. It represents 5 to 8% of all MG patients. Few pediatric cases were reported. Clinical presentation is often atypical. It is characterized by predominant involvement of cranial, bulbar, and axial muscles and early respiratory crises. Myokymia and fasciculation are suggestive of MuSK-MG. The clinical course of patients with MuSK-MG is worse than other types of MG. Responses to standard therapies are variable. We report clinical, neurophysiological, serological, and outcome profile of a Tunisian child with MuSK-MG.



Medicinus ◽  
2020 ◽  
Vol 7 (5) ◽  
pp. 150
Author(s):  
Devita Widjaja ◽  
Vivien Puspitasari

Generalized myasthenia gravis is a rare case of autoimmune wherein the antibodies destroy the post-sinaptic acetylcholine receptors at skeletal muscle’s neuromuscular junctions. The clinical presentation is specific distributin of motoric deficit without sensoric deficit which diminished with rest and worsens with excessive use. We report a case of a woman 52 yo with symptoms of ptosis, diplopia and dificulty of swallowing. Repetitive nerve stimulation showed >10% decrement and prostigmin test was positive. The patient was treated and showed clinical improvement.



Author(s):  
Kaladhar Bomma ◽  
Amaresh R. Malempati

Background: Recent advances in diagnostic and surgical techniques have brought major changes in the clinical presentation, diagnosis and the surgical management of mediastinal masses. Indian literature in this scenario is deficient, which our retrospective descriptive study aims to address.Methods: Details of patients operated for mediastinal masses from January 2007 November 2013 in two units at NIMS were collected. Clinical presentation, symptomatology, radiological/pathological findings, surgical approaches, intraoperative/immediate/intermediate post-operative results were evaluated and analysed.Results: Of the 75 patients, (48 males, range 10 years to 65 years) 2 deaths were due to complications following exacerbation of myasthenia gravis, and two patients died due to post-operative bleeding. Thymicneoplasms were the most common, followed by neurogenic tumours. Most common presentation was myasthenia gravis, followed by nonspecific back pain. 4% of patients had symptoms due to local compressive effects. Tumour was in the anterior mediastinum, middle and posterior mediastinum in 53, 16 and 6 cases respectively. Surgery done with a curative intent was through sternotomy (59), posterolateral thoracotomy (14), and combined sternotomy and cervical route (2). Residual tumour was present in 5 cases due to nerve involvement. Follow up was 98% (2 months to 6 years). 3 patients died of unrelated causes and one developed multiple lung metastases. 2 patients with neurogenic tumours developed recurrence.Conclusions: Inadequately optimized myasthenia patients and superior vena cava obstruction are poor prognostic factors. Complete excision may not be possible in neurogenic extension to spine even with concomitant neurosurgery. Neurological infiltration has a poorer prognosis.



Author(s):  
Annette Johansen ◽  
Søren Just Christensen ◽  
David Scheie ◽  
Joan L. S. Højgaard ◽  
Daniel Kondziella

Neuromuscular adverse events following cancer treatment with anti-programmed cell death protein 1 (PD-1) monoclonal antibodies are relatively rare, yet potentially fatal. Using the PRISMA approach, we performed a systematic review to characterize the clinical presentation, diagnostic workup, and management of neuromuscular disorders (NMDs) in patients treated with nivolumab or pembrolizumab. Sixty-three publications on 85 patients (mean age 66,9 years (range 34–86); male/female 2.6:1; 59% metastatic melanoma) were identified from selected indexing databases until June 2018. Forty-eight patients had received nivolumab and 39 pembrolizumab. The mean number of PD-1 inhibitor treatment cycles prior to onset of symptoms was 3,6 (range 1–28). Symptoms included oculomotor (47%); respiratory (43%), bulbar (35%), and proximal weakness (35%); as well as muscle pain (28%). Diagnoses were categorized as myasthenia gravis (27%), neuropathy (23%), myopathy (34%) and a combination of these (16%). After critical review of the data, however, evidence did not support the stated NMD diagnosis in 13% of cases, while up to 14% of patients had signs of additional NMDs. PD-1 inhibitor associated myasthenia was associated with cardiac complications in almost 30% of patients and with a more rapid clinical progression compared with idiopathic myasthenia. Mortality was high despite adequate treatment strategies including corticosteroid, IV immunoglobulins and plasmapheresis. In conclusion, clinical presentation of NMDs associated with PD-1 inhibitors is often atypical, with significant overlap between myasthenia gravis and myopathy; and cardiac/respiratory complications are common, leading to more severe disease courses than idiopathic myasthenia.



Author(s):  
Jan B. M. Kuks ◽  
Hans J. G. H. Oosterhuis


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