Myasthenia gravis in children: analysis of 18 patients
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 UNIFESP-EPM, from January 1983 to August 1997. There were 10 girls and 8...
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| Format: | Article |
| Language: | English |
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Academia Brasileira de Neurologia (ABNEURO)
2001-01-01
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| Series: | Arquivos de Neuro-Psiquiatria |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2001000500005 |
| _version_ | 1818194038327607296 |
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| author | Morita Maria da Penha A. Gabbai Alberto A. Oliveira Acary S.B. Penn Audrey S. |
| author_facet | Morita Maria da Penha A. Gabbai Alberto A. Oliveira Acary S.B. Penn Audrey S. |
| author_sort | Morita Maria da Penha A. |
| collection | DOAJ |
| description | 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 UNIFESP-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. Four 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. |
| first_indexed | 2024-12-12T00:55:56Z |
| format | Article |
| id | doaj.art-0416c8d94fa14ccfa65895c72ea13a6d |
| institution | Directory Open Access Journal |
| issn | 0004-282X 1678-4227 |
| language | English |
| last_indexed | 2024-12-12T00:55:56Z |
| publishDate | 2001-01-01 |
| publisher | Academia Brasileira de Neurologia (ABNEURO) |
| record_format | Article |
| series | Arquivos de Neuro-Psiquiatria |
| spelling | doaj.art-0416c8d94fa14ccfa65895c72ea13a6d2022-12-22T00:43:52ZengAcademia Brasileira de Neurologia (ABNEURO)Arquivos de Neuro-Psiquiatria0004-282X1678-42272001-01-01593B681685Myasthenia gravis in children: analysis of 18 patientsMorita Maria da Penha A.Gabbai Alberto A.Oliveira Acary S.B.Penn Audrey S.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 UNIFESP-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. Four 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2001000500005myasthenia gravischild |
| spellingShingle | Morita Maria da Penha A. Gabbai Alberto A. Oliveira Acary S.B. Penn Audrey S. Myasthenia gravis in children: analysis of 18 patients Arquivos de Neuro-Psiquiatria myasthenia gravis child |
| title | Myasthenia gravis in children: analysis of 18 patients |
| title_full | Myasthenia gravis in children: analysis of 18 patients |
| title_fullStr | Myasthenia gravis in children: analysis of 18 patients |
| title_full_unstemmed | Myasthenia gravis in children: analysis of 18 patients |
| title_short | Myasthenia gravis in children: analysis of 18 patients |
| title_sort | myasthenia gravis in children analysis of 18 patients |
| topic | myasthenia gravis child |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2001000500005 |
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