Myasthenia gravis in children: a longitudinal study

Ashraf, VV; Taly, Arun B.; Mustare, Veerendrakumar; Rao, Shivaji

doi:10.1111/j.1600-0404.2006.00646.x

Cited by 78 publications

(87 citation statements)

References 19 publications

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“…Their patients presented mainly with ocular symptoms (84.3%) at a mean age of 4.1 years. 22 Compared with studies describing pediatric myasthenia from China, Korea, Jamaica, Europe, and India, [23][24][25][26][27] whose populations are more ethically uniform, the current study is unique in that the Canadian population is ethnically diverse. Consequently, the differences in terms of gender, age of onset, and subtype of myasthenia gravis likely reflect differences in the ethnic backgrounds and associated genetic susceptibility between the populations.…”

Section: Discussionmentioning

confidence: 99%

Clinical Characteristics of Pediatric Myasthenia: A Surveillance Study

et al. 2013

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OBJECTIVE: To evaluate the incidence, clinical features, diagnostic, and treatment trends of pediatric myasthenia in Canada. METHODS: Through established Canadian Pediatric Surveillance Program methodology, physicians were anonymously surveyed for cases of pediatric myasthenia using a standardized clinical questionnaire containing deidentified data. Inclusion criteria were any child <18 years old with ≥1 of the following: (1) fluctuating ptosis or extraocular weakness, (2) skeletal muscle weakness or fatigue, and (3) any of the following supportive tests: clinical response to acetylcholinesterase inhibitor, positive antibodies, abnormal slow repetitive nerve stimulation, or single-fiber electromyography. RESULTS: In 2 years of surveillance, 57 confirmed cases were reported. There were 34 generalized and 18 ocular reports of juvenile myasthenia gravis plus 5 congenital myasthenic syndrome cases. There were 14 incident cases in 2010 and 6 in 2011. Age of onset ranged from “birth” to 17 years for the generalized form compared with 18 months to 11 years for the ocular subtype. Positive acetylcholine receptor titers were found in 22 (67%) of 33 generalized cases and 8 (44%) of 18 ocular patients. Of patients started on pyridostigmine, improvement was noted in 33 (100%) of 33 generalized cases and 15 (88%) of 17 ocular cases. CONCLUSIONS: This study represents the largest descriptive series of pediatric myasthenia in North America and provides valuable information about clinical characteristics. A high index of suspicion is important for this treatable disease. Children generally respond promptly to readily available therapies.

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Section: Discussionmentioning

confidence: 99%

Clinical Characteristics of Pediatric Myasthenia: A Surveillance Study

et al. 2013

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“…Ptosis is a presenting feature in most of them followed by limitation of eye movement [9]. Autoimmune MG only exceptionally starts as early as the first year of life, although seronegativity is relatively frequent in young MG patients [10,11].…”

Section: Discussionmentioning

confidence: 99%

Congenital myasthenic syndrome due to mutation in CHRNE gene

Jastrzębska¹

2017

Child Neurology

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STRESZCZENIEWrodzone zespoły miasteniczne (CMS) należą do grupy chorób rzadkich, heterogennych genetycznie. Są zaliczane do chorób złącza nerwowo -mięśniowego. Wśród CMS najczęściej występują postsynaptyczne CMS. Objawy mogą być zbliżone do objawów autoimmunologicznej miastenii, a diagnostyka opiera się na badaniach genetycznych. W naszej pracy prezentujemy przypadek chłopca z wrodzonym zespołem miastenicznym spowodowanym mutacją w genie CHRNE i z dodatnim wywiadem rodzinnym w kierunku autoimmunologicznej miastenii. Słowa kluczowe: Wrodzone zespoły miasteniczne, mutacja CHRNE, miastenia gravis ABSTRACT Congenital myasthenic syndromes (CMS) belong to a group of rare genetically heterogeneous diseases that affect neuromuscular junction. The most frequent are postsynaptic CMS. Symptoms may mimic myasthenia gravis and diagnosis relies on genetic testing. We report a case of a boy with CMS due to CHRNE mutation and positive family history of autoimmune myasthenia gravis.

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“…Bu dö-nemde, genellikle oküler kaslarda güçsüzlük ile başlar ve puberte öncesi başlayanların aksine, hastaların %80'inde 2 yıl içinde jeneralize forma dönüşür. Özellikle puberte sonrası başlayanlar-da miyastenik kriz sık görülür [1][2][3]10]. Puberte öncesinde ise, en sık 2-4 yaşlar arasında başlar ve bu dönemde kız ve erkek oranı eşittir.…”

Section: Klinikunclassified

“…Puberte öncesinde ise, en sık 2-4 yaşlar arasında başlar ve bu dönemde kız ve erkek oranı eşittir. Tutulum sıklıkla oküler kaslar ile sınırlıdır ve bü-yük çocuklara oranla daha hafif bir klinik seyir gösterir [2,10].…”

Section: Klinikunclassified

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Juvenile Myasthenia Gravis: A Report of Three Cases and Literature Review

Yılmaz¹

2015

Ann Clin Anal Med

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ÖzetAmaç: Jüvenil miyasteni gravis tüm olguların sadece %10-15'ini oluşturur ve puberte öncesi daha da nadirdir. Bu nedenle, özellikle erken yaşta başlayan miyasteni graviste, klinik tablo, tanı, tedavi ve prognoz hakkında yeterli veri yoktur. Erken tanı hastalığın progresyonu ve hayatı tehdit edebilen miyastenik krizlerin önlenmesi için önemlidir. Asetilkolin reseptörüne karşı gelişen antikorların gösterilmesi tanı için oldukça değerlidir. Ancak, yetişkin hastaların aksine, asetilkolin reseptör antikoru puberte öncesi dönemde hastaların ancak yarısında pozitif bulunur. Seronegatif hastaların, benzer klinik tablo gös-terebilen konjenital miyasteni gravisten ayrılması önemlidir. Gereç ve Yön-tem: Bu çalışmada, jüvenil miyasteni gravisli 3 olgunun, başvuru bulguları, klinik seyri ve 2 yıllık takip sonuçları sunularak ayrıntılı literatür tartışması yapılmıştır. Bulgular: Başlangıç yaşı 3, 4 ve 7 yıldı. Tüm olgularda ilk belirti gün içinde değişkenlik gösteren pitozisti. Yaşları daha küçük olan iki olguda, hastalığın başlangıcında pozitif sınırın altında olan asetilkolin reseptör antikor düzeyi bir yıl sonra yaklaşık 2 kat artarak pozitif oldu. Bu 2 hasta pridostigmin tedavisine iyi yanıt verdi ve 21 aylık izlem süresince yakınmaları olmadı. Başlangıçta asetilkolin reseptör antikor düzeyi yüksek olan diğer olgu, pridostigmin tedavisi altında hastalığın 10. ayından sonra jeneralizasyon göster-di. Prednizolon tedavisine yeterli yanıt göstermeyen hasta azatiopirin ve intravenöz immünoglobulin tedavisine iyi yanıt verdi. Tartışma: Erken çocukluk döneminde başlayan ve başlangıçta asetilkolin reseptör antikor düzeyi düşük olan jüvenil miyasteni gravis genellikle selim seyirlidir. Belirtilerin başladığı dönemde negatif olan asetilkolin reseptör antikoru, 1 yıl sonra pozitif bulunabilir. Geç çocukluk döneminde başlayan ve antikor düzeyi yüksek olan olgularda ise klinik seyir daha ağır olup immünosüpresif tedavi gerekebilir. Anahtar KelimelerJüvenil Miyasteni Gravis; Asetilkolin Reseptör Antikoru; Seronegatif Abstract Aim: Juvenile myasthenia gravis accounts for 10% to 15% of all cases and it is even rarer in prepubertal children. Therefore, limited data are available on its presentation, diagnosis, treatment and outcome, particularly in younger children. Early diagnosis is imperative for delaying the progression of the disease, and prevention and adequately management of the myasthenic crisis. Presence of acetylcholine receptor antibodies which occur only in half of prepubertal children wih myastenia helps in the diagnosis. Seronegative cases need to be distinguished from congenital forms which may present with similar symptoms. Material and Method: This article presents the initial presentation, clinical course, and 2-year follow-up results of 3 patients with juvenile myasthenia gravis, and reviewes the disease in general and current treatment modalities. Results: The ages at onset were 3, 4 and 7 years. The initial symptom was fluctuating pitosis in all cases. Acetylcholine receptor antibodies were below the upper limit o...

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Myasthenia gravis in children: a longitudinal study

Abstract: This study shows some distinctive characteristics of JMG, such as higher frequency of ocular myasthenia, benign course, better long-term outcome and lack of association of thymoma and other autoimmune disorders.

Cited by 78 publications

References 19 publications

Clinical Characteristics of Pediatric Myasthenia: A Surveillance Study

Clinical Characteristics of Pediatric Myasthenia: A Surveillance Study

Congenital myasthenic syndrome due to mutation in CHRNE gene

Juvenile Myasthenia Gravis: A Report of Three Cases and Literature Review

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