A miastenia gravis juvenil (MG) é uma forma rara de MG, uma doença autoimune da junção neuromuscular que resulta em manifestações oculares ou fraqueza generalizada, com início antes dos 18 anos de idade.
Introdução
O que você precisa saber de cara
A miastenia gravis juvenil (MG) é uma forma rara de MG, uma doença autoimune da junção neuromuscular que resulta em manifestações oculares ou fraqueza generalizada, com início antes dos 18 anos de idade.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 1 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Miastenia gravis juvenil
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Outros ensaios clínicos
Publicações mais relevantes
Juvenile Myasthenia Gravis: An Indian Perspective.
Myasthenia Gravis is an autoimmune disorder of the neuromuscular junction which is divided into three categories in children: neonatal or transient, congenital myasthenic syndromes, and autoimmune or juvenile myasthenia gravis (JMG). JMG differs from adult MG in many aspects. Characteristics of JMG also vary between children of various ethnic groups. Although common, there is scant data about JMG in India. This study was performed to determine the characteristics of JMG in Indian children. We did a retrospective chart analysis of all patients who received a diagnosis of myasthenia at a tertiary care pediatric hospital in Mumbai, India from January 2005 to December 2015. We studied the demographic data, clinical features, investigations, history of crises, and records of hospital stay. Treatment modalities and outcomes were reviewed. Fifteen children received a diagnosis of JMG during the study period. This was a pre-pubertal cohort with a mean age of 3.7 years. Nine patients had generalized symptoms at presentation and six had ocular presentation. The seropositivity rate was 73%. Patients were treated with a combination of pyridostigmine, steroids, and additional immunosuppression. Nearly half the patients needed second-line immunosuppression. Four children underwent thymectomy. MG in Indian children is a severe disease with high rates of seropositivity and a relatively better outcome if identified and treated appropriately. Thymectomy has an important role in management but requires more scrutiny.
Sport Performance as a Diagnostic and Monitoring Tool in Juvenile Myasthenia Gravis.
Pediatric Myasthenia Gravis.
Pediatric myasthenia gravis encompasses a heterogenous group of disorders that cause impaired neuromuscular junction transmission. Patients exhibit a characteristic fatigable weakness that can be life-threatening if respiration is affected. In children, myasthenia gravis can be grouped into 3 subtypes: transient neonatal myasthenia (TNM) arising from transplacental antibody transfer, congenital myasthenic syndromes (CMS) related to genetic defects, and juvenile myasthenia gravis (JMG) caused by antibodies to synaptic components. Diagnostic evaluation combines clinical evaluation with serologic and electrophysiologic studies. The condition is managed medically with acetylcholinesterase inhibitors and immunosuppressive agents. Thymectomy is an important part of the treatment of JMG, and intravenous immunoglobulin (IVIG) and plasma exchange (PLEX) play a role in acute, severe exacerbations. Management of the ophthalmic complications, including strabismus and ptosis, is necessary to prevent permanent vision loss from amblyopia.
Experience Using Efgartigimod to Treat Juvenile Myasthenia Gravis in China: A Multicenter Retrospective Study.
Current therapeutic management of juvenile myasthenia gravis (JMG) predominantly relies on conventional immunosuppressive therapies and expert consensus extrapolated from adult data, creating a critical gap in high-quality, pediatric-specific clinical evidence. The aim of this study was to assess the efficacy and safety of efgartigimod in the pediatric population. This multicenter retrospective study enrolled JMG patients (under 18 years), from 12 Chinese centers, who received at least one dose of efgartigimod. Efgartigimod was administered as weekly intravenous infusions. Study visits included W0 (baseline: pretreatment) and W1-W4 (weekly posttreatment follow-ups). Efficacy was assessed via MG-related Activities of Daily Living (MG-ADL) score, Quantitative Myasthenia Gravis (QMG) score, and the MG Composite (MGC) scale. Seventeen JMG patients (3 males, 14 females) with a median disease duration of 23 months were included. At W0, 88.2% of patients were classified as Myasthenia Gravis Foundation of America (MGFA) class II-V and were anti-acetylcholine receptor (anti-AchR) antibody positive. After efgartigimod treatment, clinically meaningful improvement (CMI) was observed in 70.6% (W1), 73% (W2), and 91.7% (W4); 66.7% achieved minimal symptom expression by W4. MG-ADL and QMG scores decreased by 30% and 30.1% (W1) and by 87.6% and 71.8% (W4) compared to W0. No treatment-related adverse events occurred. Our study suggests efgartigimod is an effective therapeutic option for JMG, with favorable efficacy and safety profiles. Further studies are necessary to validate the efficacy and safety of efgartigimod in the pediatric population.
Efficacy and safety of immunosuppressants and immunomodulators in juvenile myasthenia gravis: a systematic review and meta-analysis.
In the present meta-analysis, we aimed to explore the efficacy and safety of immunosuppressants and immunomodulators for the treatment of juvenile myasthenia gravis (JMG). We conducted a systematic search for studies published between January 1st, 2000 and July 28th, 2025, in PubMed, Embase, Web of Science, and the Cochrane Library. Statistical analyses were performed using Stata (version 16.0). Cochran's Q test and the I2 statistic were used to assess the heterogeneity among the included studies. If significant heterogeneity existed (I2 ≥50% or P < 0.05), the random effects model was used; otherwise, the fixed effects model was used to calculate the pooled results. A total of 3029 articles were retrieved. This meta-analysis included 9 cohort and case-control studies, 11 case series, 3 single-arm studies, and 1 randomized controlled trial, focusing on tacrolimus, glucocorticoids, monoclonal antibodies, and intravenous immunoglobulin. Regarding tacrolimus, 9 studies involving 310 patients assessed the efficacy of tacrolimus for treating JMG. The results showed a significant reduction in both the Quantitative Myasthenia Gravis (QMG) and Myasthenia Gravis Activities of Daily Living (MG-ADL) scores. Moreover, tacrolimus treatment allowed for a reduction in steroid dosage, with a response rate of 0.862 (95% CI: 0.716-0.967). For monoclonal antibodies, 6 studies with 67 patients analyzed the efficacy for JMG. The response rate of monoclonal antibodies was 0.993 (95% CI: 0.935-1.000). Descriptive analyses were conducted for glucocorticoids and IVIG. Besides, 5 studies with 348 patients assessed the efficacy of glucocorticoids for JMG. Included studies showed that the efficacy rate of glucocorticoid monotherapy for isolated ocular myasthenia gravis (OMG) was higher than that for patients with both OMG and generalized myasthenia gravis (GMG). Finally, regarding the use of IVIG, 4 studies reported efficacy for JMG. These investigations reported a response rate ranging from 47.06% to 94.3% for IVIG therapy. In summary, this was the first comprehensive meta-analysis of immunosuppressants and immunomodulators in JMG. However, most included studies were single-center retrospective observational studies. Future prospective multicenter studies are needed to further investigate the efficacy and safety of immunosuppressants and immunomodulators in JMG.
Publicações recentes
Centrifugal Versus Membrane-Based Therapeutic Plasma Exchange in Pediatric Seronegative Neuroimmune Disorders: Neurologic Outcome and Age Specific Safety Profile.
Pediatric Myasthenia Gravis.
Juvenile Myasthenia Gravis: An Indian Perspective.
Experience Using Efgartigimod to Treat Juvenile Myasthenia Gravis in China: A Multicenter Retrospective Study.
Efficacy and safety of immunosuppressants and immunomodulators in juvenile myasthenia gravis: a systematic review and meta-analysis.
📚 EuropePMC133 artigos no totalmostrando 85
Pediatric Myasthenia Gravis.
International ophthalmology clinicsJuvenile Myasthenia Gravis: An Indian Perspective.
Neurology IndiaExperience Using Efgartigimod to Treat Juvenile Myasthenia Gravis in China: A Multicenter Retrospective Study.
Muscle & nerveEfficacy and safety of immunosuppressants and immunomodulators in juvenile myasthenia gravis: a systematic review and meta-analysis.
Journal of translational medicineImproved Neuromuscular Transmission Following Thymectomy in Juvenile Myasthenia Gravis: A Case Series.
Muscle & nerveCorticosteroid-Refractory Juvenile Myasthenia Gravis: Treatment Responses and Prognosis in a Large Chinese Cohort.
Muscle & nerveSport Performance as a Diagnostic and Monitoring Tool in Juvenile Myasthenia Gravis.
Journal of child neurologyPerioperative Management of Minimally Invasive Thymectomy in a Pediatric Patient With Myasthenia Gravis After Respiratory Crisis: A Case Report.
A&A practicePersonalized Management and Timing of Thymectomy in Juvenile Myasthenia Gravis: Insights from Routine Clinical Scale Use in a Single-Center Retrospective Cohort-A Case Series.
Children (Basel, Switzerland)Robot-assisted extended thymectomy in juvenile myasthenia gravis: first case performed in Ecuador.
Journal of surgical case reportsMyasthenia Gravis as a Manifestation of Aicardi-Goutières Syndrome Due to a SAMHD1 Variant Successfully Treated With Baricitinib.
CureusA Rare Case of Juvenile Myasthenia Gravis With Low-Density Lipoprotein Receptor-Related Protein 4 Antibodies in a Seven-Year-Old Boy.
Pediatric neurologyJuvenile Myasthenia Gravis Simulating Bell's Palsy in a 3-year-old Female - A Unique Clinical Presentation.
International journal of applied & basic medical researchTreatment Patterns and Disease Burden of Juvenile Myasthenia Gravis in the United States: A Cohort Study Using Health Care Claims Databases.
NeurologyIncidence and Prevalence of Juvenile Myasthenia Gravis in the United States Between 2010 and 2020: Analysis of Two Claims Databases.
Neurology and therapyA Juvenile Myasthenia Gravis Crisis in a Previously Undiagnosed Child-A Case Report.
Journal of paediatrics and child healthEpidemiology and 10-year clinical care of juvenile myasthenia gravis in England: a retrospective cohort study.
BMJ neurology openEfficacy and Safety of Tacrolimus Therapy in Patients With Juvenile Myasthenia Gravis: A Single-Arm Meta-Analysis.
Pediatric neurology275th ENMC international workshop: Seronegative myasthenia gravis: An update paradigm for diagnosis and management, 9-11 February 2024, Hoofddorp, the Netherlands.
Neuromuscular disorders : NMDLipoprotein Receptor-Related Protein 4 Antibody Positivity in the Youngest Patient in the Caucasus Region: A Case Report.
CureusTreatment of juvenile myasthenia gravis with tacrolimus: A cohort study.
European journal of neurologyTargeted Treatments for Myasthenia Gravis in Children and Adolescents.
Paediatric drugsImmune mediated myasthenia gravis in children, current concepts and new treatments: A narrative review article.
Iranian journal of child neurologyThymectomy for juvenile myasthenia gravis: a narrative review.
Mediastinum (Hong Kong, China)Clinical Characteristics and Treatment of Juvenile Myasthenia Gravis-A Single-Center Experience.
Children (Basel, Switzerland)Juvenile Myasthenia Gravis in North Texas: Clinical Features, Treatment Response, and Outcomes.
Pediatric neurology[Treatment of Juvenile Myasthenia Gravis].
Brain and nerve = Shinkei kenkyu no shinpoThe Clinical Course and Treatment of a Case of Refractory Systemic Juvenile Myasthenia Gravis Successfully Treated with Thymectomy.
The Tohoku journal of experimental medicinePediatric Neuromuscular Diseases.
Pediatric neurologyGenetic, serological and clinical evaluation of childhood myasthenia syndromes- single center subgroup analysis experience in Turkey.
Acta neurologica BelgicaMeasuring the Efficacy of Thymectomy for Pediatric Myasthenia Gravis Across Tertiary Children's Hospitals.
Pediatric neurologyOutcome and clinical features in juvenile myasthenia gravis: A systematic review and meta-analysis.
Frontiers in neurologyJuvenile Myasthenia Gravis in a 14-year-old adolescent masked by mood disorder: the complex balance between neurology and psychiatry.
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of MyologyEssen transition model for neuromuscular diseases.
Neurological research and practiceRituximab in juvenile myasthenia gravis-an international cohort study and literature review.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society[Analysis of clinical characteristics and related genetic variation of juvenile myasthenia gravis].
Zhonghua yi xue za zhiDemographics and ocular findings in children with myasthenia.
Eye (London, England)Effect of Two Different Rehabilitation Approaches on Pulmonary Functional Tests, Neuromuscular Functions and Quality of Life in Juvenile Myasthenia Gravis: A Randomized Controlled Trial Study.
Medicina (Kaunas, Lithuania)Rituximab Therapy in the Treatment of Juvenile Myasthenia Gravis: The French Experience.
NeurologyThe Epidemiology and Phenotypes of Ocular Manifestations in Childhood and Juvenile Myasthenia Gravis: A Review.
Frontiers in neurology[Essen transition model for neuromuscular diseases].
Der NervenarztSpontaneous remission in juvenile myasthenia gravis: A cohort of 13 cases and review of the literature.
Neuromuscular disorders : NMDMyasthenia Gravis Masquerading as Status Asthmaticus.
Case reports in pediatricsEffectiveness of thymectomy in juvenile myasthenia gravis and clinical characteristics associated with better outcomes.
Neuromuscular disorders : NMDJuvenile Generalized Myasthenia Gravis: Presented as Unilateral Blepharoptosis and Successfully Managed with Pulse Intravenous Methylprednisolone.
Kathmandu University medical journal (KUMJ)[Surgical results of video-assisted thoracoscopic thymectomy for treatment of Juvenile Myasthenia Gravis].
Andes pediatrica : revista Chilena de pediatriaNonorganic visual loss in a child.
Saudi journal of ophthalmology : official journal of the Saudi Ophthalmological SocietyThymectomy in Juvenile Myasthenia Gravis Is Safe Regarding Long Term Immunological Effects.
Frontiers in neurologyExtended thymectomy via subxiphoid uniportal Video-Assisted Thoracoscopic Surgery: A case report.
International journal of surgery case reportsDeveloping outcome measures of disease activity in pediatric myasthenia.
Muscle & nerveCorrigendum to "Workshop report: 242nd ENMC international workshop: Diagnosis and management of juvenile myasthenia gravis, Hoofddorp, the Netherlands, 1-3 March 2019" [Neuromuscular Disorders 30 (2020) 254-264].
Neuromuscular disorders : NMDOutcomes of Juvenile Myasthenia Gravis: A Comparison of Robotic Thymectomy With Medication Treatment.
The Annals of thoracic surgeryRituximab as Adjunct Maintenance Therapy for Refractory Juvenile Myasthenia Gravis.
Pediatric neurologyManagement of Juvenile Myasthenia Gravis.
Frontiers in neurologyWhat's in the Neuromuscular Junction Literature?
Journal of clinical neuromuscular disease242nd ENMC International Workshop: Diagnosis and management of juvenile myasthenia gravis Hoofddorp, the Netherlands, 1-3 March 2019.
Neuromuscular disorders : NMDOpen versus thoracoscopic thymectomy for juvenile myasthenia gravis.
Journal of pediatric surgeryPediatric Ocular Myasthenia Gravis.
Current treatment options in neurology"Mummy, my eyelids are heavy": A case series of juvenile myasthenia gravis.
European journal of ophthalmologyCorrelation of anti-acetylcholine receptor antibody levels and long-term outcomes of juvenile myasthenia gravis in Taiwan: a case control study.
BMC neurologyCell-Based Versus Enzyme-Linked Immunosorbent Assay for the Detection of Acetylcholine Receptor Antibodies in Chinese Juvenile Myasthenia Gravis.
Pediatric neurologyThoracoscopic thymectomy for juvenile myasthenia gravis.
Pediatric surgery internationalIncidence and Ocular Features of Pediatric Myasthenias.
American journal of ophthalmology[Peripheral nervous system immunological disorder].
MedicinaSerial Stimulated Jitter Analysis In Juvenile Myasthenia Gravis.
Muscle & nerveClinical Characteristics of Juvenile Myasthenia Gravis in Southern China.
Frontiers in neurology[Juvenile myasthenia gravis in sub-Saharan Africa: a case study of two consanguine sisters born from consanguinity in Togo].
The Pan African medical journalJuvenile myasthenia gravis in Norway: HLA-DRB1*04:04 is positively associated with prepubertal onset.
PloS oneClinical features and evolution of juvenile myasthenia gravis in a French cohort.
Muscle & nerveJuvenile myasthenia gravis.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology SocietyOutcome after Robotic-Assisted Thymectomy in Children and Adolescents with Acetylcholine Receptor Antibody-Positive Juvenile Myasthenia Gravis.
NeuropediatricsAnti-MuSK-Positive Myasthenic Crisis in a 7-Year-Old Female.
Case reports in emergency medicineJuvenile myasthenia gravis in Norway: Clinical characteristics, treatment, and long-term outcome in a nationwide population-based cohort.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology SocietyThe role of thymectomy in the treatment of juvenile myasthenia gravis: a systematic review.
Pediatric surgery internationalEarly onset bilateral juvenile myasthenia gravis masquerading as simple congenital ptosis.
GMS ophthalmology cases[The pitfalls of using diagnosis and treatment guidelines for patients with juvenile myasthenia gravis].
No to hattatsu = Brain and developmentJuvenile myasthenia gravis in Norway: A nationwide epidemiological study.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology SocietyJuvenile Myasthenia Gravis in Korea: Subgroup Analysis According to Sex and Onset Age.
Journal of child neurologyMultidisciplinary treatment for prepubertal juvenile myasthenia gravis with crisis.
Pediatrics international : official journal of the Japan Pediatric SocietyOutcomes of thoracoscopic thymectomy in patients with juvenile myasthenia gravis.
Journal of pediatric surgery[Effects of thymectomy on bone age and height development in juvenile myasthenia gravis].
Zhonghua yi xue za zhiThe African-387 C>T TGFB1 variant is functional and associates with the ophthalmoplegic complication in juvenile myasthenia gravis.
Journal of human geneticsResults of surgical treatment for juvenile myasthenia gravis.
Neurologia (Barcelona, Spain)[Clinical characteristics and managements of juvenile myasthenia gravis].
Nihon rinsho. Japanese journal of clinical medicineCo-existence of juvenile-onset systemic lupus erythematosus and juvenile myasthenia gravis.
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Juvenile Myasthenia Gravis: An Indian Perspective.
- Sport Performance as a Diagnostic and Monitoring Tool in Juvenile Myasthenia Gravis.
- Pediatric Myasthenia Gravis.
- Experience Using Efgartigimod to Treat Juvenile Myasthenia Gravis in China: A Multicenter Retrospective Study.
- Efficacy and safety of immunosuppressants and immunomodulators in juvenile myasthenia gravis: a systematic review and meta-analysis.
- Centrifugal Versus Membrane-Based Therapeutic Plasma Exchange in Pediatric Seronegative Neuroimmune Disorders: Neurologic Outcome and Age Specific Safety Profile.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:391497(Orphanet)
- MONDO:0018325(MONDO)
- Miastenia Gravis(PCDT · Ministério da Saúde)
- GARD:21624(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55787956(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
