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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.47 no.2 São Paulo June 1989

http://dx.doi.org/10.1590/S0004-282X1989000200019 

Miastenia grave familiar: relato de caso em gêmeas idênticas

 

Familial myasthenia gravis: case report in identical twins

 

 

Elza Dias-TostaI; Maria de Fátima Machado AguiarII; Hamilton BarbosaII; Suzete Silva Leme VilelaII

INeurologista do Hospital de Base do Distrito Federal (HBDF), PhD
IINeurologista da Unidade de Pediatria do HBDF

 

 


RESUMO

Trata-se do sétimo relato da literatura mundial de casos de miastenia gravis em gêmeos homozigóticos em que ambos são acometidos. O homozigotismo foi provado com certeza por estudo de HLA e a forma adquirida da doença foi provada por determinação de níveis elevados de anticorpos anti-receptor de acetilcolina, havendo também níveis elevados de anticorpos antimúsculo estriado, sem outras evidências de timoma.


SUMMARY

To the best of our knowledge this is the seventh case report of monozygotic twins both affected by myasthenia gravis (MG). The monozygotism was proven by sex identity, blood group and HLA determinations ('O' Rh +, A2, A19.2, B40, CW3). One paternal aunt was also affected and the three cases have high titles of anti-acetylcholine receptor antibodies and anti-striated muscle antibodies, which indicate an acquired form of MG. After several myasthenic crises the twins are now doing well with corticosteroid therapy. The paternal aunt has a more benign form of MG, with ocular and limb involvement (grade IIa of Osserman) and was submitted to thymectomy. The authors discuss the use of corticosteroid for the infantile form of MG instead of thymectomy, based on the immaturity of the immune system in the young age. The use of other immunosupressive drugs is not advisable because of potential hazardous development of neoplasms.


 

 

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

 

 

Agradecimentos - Dr. Ricardo Manoel Oliveira, do Laboratório de Investigação em Reumatologia do HC da USP, que dosou os anticorpos; Dr. Antônio Vilaça, que tipou o HLA (HDA, Brasília); Dra. Maria Helena Chagas responsável pelos estudos neurofisiológicos (HBDF, Brasília); Dr. Paulo Henrique Costa, que realizou os estudos histoquímicos (Hospital Sarah Kubitschek, Brasília).

 

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