Long‐Term Follow‐up Study of Relapse in Symptoms and Reelevation of Acetylcholine Receptor Antibody Titers in Patients with Myasthenia Gravisa

Komiya, Tadatoshi; Satô, Takeshi

doi:10.1111/j.1749-6632.1988.tb27187.x

Cited by 6 publications

(3 citation statements)

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“…We have also found that the AchRAb titers did not correlate with the severity of the disease. Like others [24][25][26] , we may speculate that lower AchRAb titers in more severely affected patients may simply reflect their more aggressive immunosuppressive treatments.…”

Section: Discussionsupporting

confidence: 52%

Myasthenia gravis in children: analysis of 18 patients

Morita

Gabbai

Oliveira

et al. 2001

Arq. Neuro-Psiquiatr.

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-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 UNI.ESP-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. .our 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.KEY WORDS: myasthenia gravis, child. Miastenia gravis na infância: estudo de 18 pacientesRESUMO -A miastenia gravis (MG) em crianças é rara e perfaz 10 a 20% dos pacientes com a doença. Relatamos 18 pacientes cujo início dos sintomas ocorreu até os 12 anos de idade, acompanhados na Disciplina de Neurologia da UNI.ESP-EPM de janeiro de 1983 a agosto de 1997. Eram 10 meninas e 8 meninos (1,2:1,0). Doença generalizada moderada ou grave ocorreu em 11 crianças (61%) e 4 (22%) tiveram pelo menos uma crise miastênica. A eletroneuromiografia com estimulação repetitiva foi diagnóstica em 8 (47%) de 17 pacientes e a tomografia computadorizada do tórax não demonstrou aumento do timo em 14 pacientes. A positividade para o anticorpo anti-receptor da acetilcolina foi de 81,6% nos 11 pacientes testados e seu nível não se correlacionou com a gravidade dos sintomas. Nove dos 16 pacientes (56%) acompanhados apresentou evolução com piora tendo sido utilizada prednisona e 4 destes (25%) pioraram apesar desta terapia e foram submetidos a timectomia. Das quatro pacientes timectomizadas (todas com hiperplasia folicular linfóide do timo), 3 (75%) melhoraram e uma (25%) continuou piorando tendo sido medicada com imunoglobulina endovenosa e azatioprina. O tratamento da MG deve ser individualizado para cada criança para chegar-se o mais próximo de uma atividade de vida diária plena.

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Section: Discussionsupporting

confidence: 52%

Myasthenia gravis in children: analysis of 18 patients

Morita

Gabbai

Oliveira

et al. 2001

Arq. Neuro-Psiquiatr.

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“…The author, in a more extensive survey (25), has already reported on this feature and has also thoroughly discussed studies in disagreement with such a finding. An upward trend of anti-AChR antibodies in sera from M G patients after thymomectomy has, in addition to the present survey (Figs 1, 2), been observed in three other studies (14,41,42). A possible explanation might be that the neoplastic thymus gland d o in fact have immunmodulatory properties with a predominance of inhibitory mechanisms, which is also consistent with "silent" thymomas, with the association between other diseases of presumed autoimmune origin and thymoma, and with the onset or exacerbation of M G after the removal of such a tumour.…”

Section: A Chr-related Determinants Located In the Thymus -supporting

confidence: 90%

Exacerbation of myasthenia gravis after removal of thymomas

Somnier

2009

Acta Neurologica Scandinavica

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It appeared from this prospective and non‐randomized study, that the removal of thymomas in myasthenia gravis (MG) patients resulted in rapid exacerbation of the clinical severity of the disease and of anti‐acetylcholine receptor antibodies titres, which peaked after about 300 days and continued for up to 2 years. Long‐term follow‐up after thymomectomy (mean duration ± SEM after surgery 5.5 ± 0.8 years) showed that the immunological and clinical state observed prior to surgery was eventually restored, but long‐term benefit attributable to surgery could not be demonstrated. Non‐thymoma MG cases, however, exhibited post‐operative amelioration in clinical course and decreasing antibodies titres, both of which were already significant one year after surgery, and additional improvement was observed at the time of long‐term follow‐up (mean 4.3 ± 0.5 years). Furthermore, the prognosis for MG patients not operated on was also favourable. It is suggested that the occurrence of thymomas is linked to genetic factors and that neoplasia of the thymus may be part of immunoregulatory mechanisms with predominance of inhibition.

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“…1 MG is caused by helper T-cell-dependent autoantibodies against muscle nicotinic acetylcholine receptor (AChR), the titer of which parallels the individual clinical course of MG patients, [2][3][4] although about 15% of patients with generalized MG are ''seronegative''. 5,6 A role for the thymus in the etiopathogenesis of MG has been strongly suggested by the high incidence of thymic abnormalities.…”

Section: Introductionmentioning

confidence: 99%