Humoral Pathogenesis of Myasthenia Grayis<sup>a</sup>

Drachman, Daniel B.; Silva, Shari de; Ramsay, David A.; Pestronk, Alan

doi:10.1111/j.1749-6632.1987.tb51285.x

Cited by 102 publications

(51 citation statements)

References 57 publications

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“…In the example of MG, approximately 15% of patients with generalized myasthenic weakness lack detectable anti-muscle AChR antibodies (1). Nevertheless, their serum immunoglobulins bind to AChR-expressing mammalian muscle cultures (7), and induce a reduction in AChRs upon passive transfer to mice (8)(9)(10). It has recently been shown that sera from approximately 40% of these patients have antibodies that bind to muscle specific kinase (MuSK), a protein that is closely linked to AChR, and plays a role in the clustering of AChRs during development of neuromuscular junctions (11).…”

Section: Five Criteria For Recognizing Antibody-mediated Autoimmune Dmentioning

confidence: 99%

“…In addition, throughout much of life, small islets continue to develop from pancreatic ducts through neogenesis and proliferation. Islet mass turnover in rodents is slow and is believed to derive from two sources: replicating β cells in pancreatic islets and neogenesis from pancreatic ducts (8). The capacity of β cells to replicate is certainly important in the postnatal period, but may be more limited at later stages in life.…”

Section: Pancreatic Islet Development From Gut Endodermmentioning

confidence: 99%

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Autonomic “myasthenia”: the case for an autoimmune pathogenesis

Drachman¹

2003

J. Clin. Invest.

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Section: Five Criteria For Recognizing Antibody-mediated Autoimmune Dmentioning

confidence: 99%

Section: Pancreatic Islet Development From Gut Endodermmentioning

confidence: 99%

Autonomic “myasthenia”: the case for an autoimmune pathogenesis

Drachman¹

2003

J. Clin. Invest.

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“…antibodies to the nicotinic acetylcholine receptor (AAChR) play an important role in the pathogenesis'- 4,8 . The clinical forms are: severe, which patients have respiratory involvement; accentuated, patients with bulbar symptoms without respiratory involvement; moderate, with only a motor dysfunction.…”

Section: Acquired Myasthenia Gravis (Mg) Is a Disease Of Neuromusculamentioning

confidence: 99%

Evaluation of the respiratory function in myasthenia gravis: an important tool for clinical feature and diagnosis of the disease

Saraiva

Assis²,

Marchiori³

1996

Arq. Neuro-Psiquiatr.

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-Myasthenic gravis may affect both inspiratory and expiratory muscles. Respiratory involvement occurred in almost all patients with myasthenia gravis in all clinical forms of the disease: 332 lung function tests done in 324 myasthenic patients without respiratory symptoms (age 34.6 ± 18.3 years) were examined. Lung volumes analysis showed that all the patients of both sexes with generalized or ocular myasthenia gravis showed "myasthenic pattern". Male patients with "ocular" form only presented the "myasthenic pattern" with lung impairment and had, from the lung function point of view, a more benign behaviour. Female patients with the "ocular" form exhibited a behaviour of respiratory variables similar to that of the generalized form. It was not observed modification of the variables that suggested obstruction of the higher airways. The "myasthenic pattern" was rarely observed in other neuromuscular diseases, except in patients with laryngeal stenosis.KEY WORDS: myasthenia gravis, lung function tests, myasthenic pattern. Avaliação da função respiratória na miastenia gravis: importância na caracterização clínica e no diagnóstico da doençaRESUMO -O comprometimento respiratório é fator limitante na evolução clinica da miastenia gravis (MG) e as formas clínicas mais graves apresentavam acometimento bulbar e respiratório. Para avaliar a reserva respiratória foram examinados em 324 pacientes com MG (forma ocular 62, generalizada 246 e timomatosa 16) as seguintes variáveis da prova de função pulmonar (PFP): capacidade vital forçada (FVC); volume onde o fluxo expiratório é igual a 1 litro por segundo (VF=1); volume expiratório forçado no primeiro segundo (FEV1); fluxo expiratório forçado medido entre 0,2 e 1,2 litros (FEF); fluxo médio expiratório forçado, medido entre 25 e 75% da FVC (FMF); intervalo de tempo entre 25 e 75% da FVC (FMFT); tempo médio de trânsito na expiração forçada (MTT); capacidade pulmonar total (TLC); volume residual (RV); curva fluxo-volume para pesquisa do "padrão miastênico". A análise estatística realizada foi: "t pareado" entre paciente e seu padrão e "t não pareado" entre grupos. Conclusões: Todos os pacientes apresentaram o padrão miastênico e esta alteração da curva fluxo-¬ volume sugeriu disfunção dos músculos da laringe. Nos pacientes com formas clinicamente localizadas as PFP também se mostraram alteradas revelando a generalização da sintomatologia mais frequentemente no sexo feminino. Não foi observada modificação das variáveis que indicam obstrução das vias aéreas decorrente do uso de anticolinesterásicos no tratamento da MG nem aumento de incidência de asma brônquica com o uso de drogas anticolinesterásicas. PALAVRAS-CHAVE: miastenia grave, provas funcionais respiratórias, padrão miastênico. Acquired myasthenia gravis (MG) is a disease of neuromuscular transmission in which antibodies to the nicotinic acetylcholine receptor (AAChR) play an important role in the

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“…Reduction in the number of active receptors at the endplate is brought about either by functional block, by an increased rate of receptor degradation, or by complement-mediated lysis of the postsynaptic membrane. 2 Further support for the concept of postsynaptic abnormality in myasthenia gravis comes from electron microscopic studies of neuromuscular junctions of myasthenic muscles. The postsynaptic membrane shows sparse, shallow folds with markedly simplified geometric patterns.l~ l Recently, it has been reported that the AChRs at normally innervated neuromuscular junctions are composed of two subpopulations with strikingly different rates of turnover.…”

Section: Neuromuscular Junctionmentioning

confidence: 99%