scholarly journals Myasthenia gravis in children: analysis of 18 patients

2001 ◽  
Vol 59 (3B) ◽  
pp. 681-685 ◽  
Author(s):  
Maria da Penha A. Morita ◽  
Alberto A. Gabbai ◽  
Acary S.B. Oliveira ◽  
Audrey S. Penn
Keyword(s):  
Myasthenia Gravis ◽  
Intravenous Immunoglobulin ◽  
Acetylcholine Receptor ◽  
Nerve Stimulation ◽  
Chest Ct ◽  
Clinical Severity ◽  
Myasthenic Crisis ◽  
Repetitive Nerve Stimulation ◽  
Follicular Hyperplasia ◽  
Receptor Antibodies

Myasthenia gravis (MG) in childhood is rare comprising 10 to 20 % of all myasthenic patients. We studied 18 patients with MG whose first symptoms started from 1 to 12 years of age, followed at the Department of Neurology of the UNIFESP-EPM, from January 1983 to August 1997. There were 10 girls and 8 boys (1.2:1). Eleven patients (61%) presented moderate or severe generalized disease and 4 (22%) had at least one myasthenic crisis. EMG with supramaximal repetitive nerve stimulation was diagnostic in 8 (47%) out of 17 patients, and chest CT was normal in 14 patients. Seropositivity to acetylcholine receptor antibodies was found in 81.6% (9 out of 11 tested) and the levels had no relation to clinical severity. Nine out of 16 patients (56%) worsened with pyridostigmine alone and were treated with prednisone. Four out of those nine continued worsening despite steroids and were subjected to thymectomy (all showed thymic lymphoid follicular hyperplasia). Three patients (75%) improved markedly after thymectomy and one (25%) worsened, eventually getting better with intravenous immunoglobulin and oral azathioprine. MG treatment, using all resources available, has to be individualized for each child.

Therapeutic Immunoadsorption of Acetylcholine Receptor Antibodies in Myasthenia Gravis

1988 ◽  
Vol 540 (1 Advances in N) ◽  
pp. 554-556 ◽  
Author(s):  
TAKESHI SATO ◽  
YASUNORI ISHIGAKI ◽  
TADATOSHI KOMIYA ◽  
HIROSHI TSUDA
Keyword(s):  
Myasthenia Gravis ◽  
Acetylcholine Receptor ◽  
Receptor Antibodies

Subunit specificity of the acetylcholine receptor antibodies in double seropositive myasthenia gravis

2021 ◽  
Author(s):  
Vasiliki Zouvelou ◽  
Maria Michail ◽  
Maria Belimezi ◽  
Paraskevi Zisimopoulou
Keyword(s):  
Myasthenia Gravis ◽  
Acetylcholine Receptor ◽  
Receptor Antibodies

Repetitive nerve stimulation: Relative muscles sensitivity in generalized myasthenia gravis

2018 ◽  
Vol 48 (4) ◽  
pp. 249
Author(s):  
Rania Zouari ◽  
Olfa Hdiji ◽  
Hanen Haj Kacem ◽  
Nadia Bouattour ◽  
Nouha Farhat ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Nerve Stimulation ◽  
Repetitive Nerve Stimulation

scholarly journals Detecting myasthenia gravis as a cause of unclear dysphagia with an endoscopic tensilon test

2021 ◽  
Vol 14 ◽  
pp. 175628642110355
Author(s):  
Tobias Warnecke ◽  
Sun Im ◽  
Bendix Labeit ◽  
Olga Zwolinskaya ◽  
Sonja Suntrup-Krüger ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Sensitivity And Specificity ◽  
Nerve Stimulation ◽  
Serum Antibody ◽  
Significant Heterogeneity ◽  
Swallowing Function ◽  
Repetitive Nerve Stimulation ◽  
Muscle Involvement ◽  
Pharyngeal Dysphagia ◽  
Tensilon Test

Aims: The flexible endoscopic evaluation of swallowing-tensilon test (FTT) was developed to diagnose myasthenia gravis (MG) in patients with unclear pharyngeal dysphagia. The purpose of this study was to determine sensitivity and specificity of the FTT and compare its diagnostic validity with that of other diagnostic markers. Methods: In this single-centre pragmatic clinical cohort study, a total of 100 patients with unclear pharyngeal dysphagia were eligible to undergo FTT. All patients were subjected to FTT and subsequently followed up clinically. FTT was considered positive if a significant improvement of pharyngeal swallowing function could be objectified endoscopically upon administration of edrophonium chloride. In addition, repetitive nerve stimulation test and serum MG antibody analysis were conducted. Results: All subjects (mean age 62.5 ± 14.1 years, female 33) underwent FTT without any complications. According to the results of the diagnostic procedures and based on long-term clinical follow-up for at least 3 years, 51 patients were finally diagnosed with MG. The sensitivity and specificity for the FTT was 88.2% and 95.9%, respectively. Application of the Cochran’s Q test showed statistically significant heterogeneity among the diagnostic tests, with results indicating FTT performance to be more accurate than the repetitive nerve stimulation results ( p < 0.001) and comparable with serum antibody tests ( p > 0.99). Conclusion: FTT has excellent clinical properties to be used routinely in the assessment of dysphagia with isolated or predominant pharyngeal muscle involvement allowing rapid and accurate diagnosis of MG.

Sign in / Sign up

Export Citation Format

Share Document