Duchenne muscular dystrophy: alpha-dystroglycan immunoexpression in skeletal muscle and cognitive performance Distrofia muscular de Duchenne: imunoexpressão da alfa-distroglicana em musculatura esquelética e performance cognitiva
The Duchenne muscular systrophy (DMD) is a muscular dystrophy with cognitive impairment present in 20-30% of the cases. In the present study, in order to study the relationship between the alpha-dystroglycan (alpha-DG) immunostaining in skeletal muscle and cognitive performance in DMD patients, 19 w...
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| Format: | Article |
| Language: | English |
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Academia Brasileira de Neurologia (ABNEURO)
2005-12-01
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| Series: | Arquivos de Neuro-Psiquiatria |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2005000600015 |
| _version_ | 1818902413429440512 |
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| author | Conceição Campanario da Silva Pereira Beatriz Hitomi Kiyomoto Ricardo Cardoso Acary Souza Bulle Oliveira |
| author_facet | Conceição Campanario da Silva Pereira Beatriz Hitomi Kiyomoto Ricardo Cardoso Acary Souza Bulle Oliveira |
| author_sort | Conceição Campanario da Silva Pereira |
| collection | DOAJ |
| description | The Duchenne muscular systrophy (DMD) is a muscular dystrophy with cognitive impairment present in 20-30% of the cases. In the present study, in order to study the relationship between the alpha-dystroglycan (alpha-DG) immunostaining in skeletal muscle and cognitive performance in DMD patients, 19 were assessed. Twelve patients performed the intelligence quotient (IQ) below the average. Among the 19 patients, two were assessed by the Stanford-Binet test and 17 by Wechsler Intelligence Scale for Children-III (WISC-III). Nine patients performed a verbal IQ below the average, only three patients performed an average verbal IQ. The muscle biopsies immunostained with antibodies to alpha-DG showed that 17 patients presented a low expression, below 25% of the total fibers. Two patients presented alpha-DG immunostaining above 40% and an IQ within the average. No significant statistical relationship was demonstrated among total IQ, verbal IQ and execution IQ and alpha-DG immunostaining at these patients muscle samples.<br>A distrofia muscular de Duchenne (DMD) é uma distrofia muscular com comprometimento cognitivo presente em 20-30% dos casos. No presente estudo, com a finalidade de estudar a relação entre a imunoexpressão da alfa-distroglicana (alfa-DG) em musculatura esquelética e a performance cognitiva em pacientes com DMD, foram avaliadas 19 crianças. Doze pacientes apresentaram o quociente de inteligência (QI) abaixo da média. Entre os 19 pacientes, dois foram avaliados pelo teste de Stanford-Binet e 17 pelo Wechsler Intelligence Scale para crianças-III (WISC-III). Nove apresentaram QI verbal abaixo da média, e apenas três QI verbal na média. As biopsias musculares com os anticorpos para alfa-DG mostraram que 17 pacientes apresentaram baixa expressão, abaixo de 25% do total de fibras. Dois pacientes apresentaram a imunoexpressão da alfa-DG acima de 40% e QI dentro da média. Não foi demonstrada relação estatisticamente significante entre o QI total, QI verbal e QI de execução e a imunoexpressão da alfa-DG . |
| first_indexed | 2024-12-19T20:35:15Z |
| format | Article |
| id | doaj.art-c6b76ef74ec2408698b50c256cd77a20 |
| institution | Directory Open Access Journal |
| issn | 0004-282X 1678-4227 |
| language | English |
| last_indexed | 2024-12-19T20:35:15Z |
| publishDate | 2005-12-01 |
| publisher | Academia Brasileira de Neurologia (ABNEURO) |
| record_format | Article |
| series | Arquivos de Neuro-Psiquiatria |
| spelling | doaj.art-c6b76ef74ec2408698b50c256cd77a202022-12-21T20:06:34ZengAcademia Brasileira de Neurologia (ABNEURO)Arquivos de Neuro-Psiquiatria0004-282X1678-42272005-12-0163498498910.1590/S0004-282X2005000600015Duchenne muscular dystrophy: alpha-dystroglycan immunoexpression in skeletal muscle and cognitive performance Distrofia muscular de Duchenne: imunoexpressão da alfa-distroglicana em musculatura esquelética e performance cognitivaConceição Campanario da Silva PereiraBeatriz Hitomi KiyomotoRicardo CardosoAcary Souza Bulle OliveiraThe Duchenne muscular systrophy (DMD) is a muscular dystrophy with cognitive impairment present in 20-30% of the cases. In the present study, in order to study the relationship between the alpha-dystroglycan (alpha-DG) immunostaining in skeletal muscle and cognitive performance in DMD patients, 19 were assessed. Twelve patients performed the intelligence quotient (IQ) below the average. Among the 19 patients, two were assessed by the Stanford-Binet test and 17 by Wechsler Intelligence Scale for Children-III (WISC-III). Nine patients performed a verbal IQ below the average, only three patients performed an average verbal IQ. The muscle biopsies immunostained with antibodies to alpha-DG showed that 17 patients presented a low expression, below 25% of the total fibers. Two patients presented alpha-DG immunostaining above 40% and an IQ within the average. No significant statistical relationship was demonstrated among total IQ, verbal IQ and execution IQ and alpha-DG immunostaining at these patients muscle samples.<br>A distrofia muscular de Duchenne (DMD) é uma distrofia muscular com comprometimento cognitivo presente em 20-30% dos casos. No presente estudo, com a finalidade de estudar a relação entre a imunoexpressão da alfa-distroglicana (alfa-DG) em musculatura esquelética e a performance cognitiva em pacientes com DMD, foram avaliadas 19 crianças. Doze pacientes apresentaram o quociente de inteligência (QI) abaixo da média. Entre os 19 pacientes, dois foram avaliados pelo teste de Stanford-Binet e 17 pelo Wechsler Intelligence Scale para crianças-III (WISC-III). Nove apresentaram QI verbal abaixo da média, e apenas três QI verbal na média. As biopsias musculares com os anticorpos para alfa-DG mostraram que 17 pacientes apresentaram baixa expressão, abaixo de 25% do total de fibras. Dois pacientes apresentaram a imunoexpressão da alfa-DG acima de 40% e QI dentro da média. Não foi demonstrada relação estatisticamente significante entre o QI total, QI verbal e QI de execução e a imunoexpressão da alfa-DG .http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2005000600015distrofia muscular de Duchennealfa-distroglicanadéficit cognitivodistrofinaDuchenne muscular dystrophyalpha-dystroglycancognitive impairmentdystrophin |
| spellingShingle | Conceição Campanario da Silva Pereira Beatriz Hitomi Kiyomoto Ricardo Cardoso Acary Souza Bulle Oliveira Duchenne muscular dystrophy: alpha-dystroglycan immunoexpression in skeletal muscle and cognitive performance Distrofia muscular de Duchenne: imunoexpressão da alfa-distroglicana em musculatura esquelética e performance cognitiva Arquivos de Neuro-Psiquiatria distrofia muscular de Duchenne alfa-distroglicana déficit cognitivo distrofina Duchenne muscular dystrophy alpha-dystroglycan cognitive impairment dystrophin |
| title | Duchenne muscular dystrophy: alpha-dystroglycan immunoexpression in skeletal muscle and cognitive performance Distrofia muscular de Duchenne: imunoexpressão da alfa-distroglicana em musculatura esquelética e performance cognitiva |
| title_full | Duchenne muscular dystrophy: alpha-dystroglycan immunoexpression in skeletal muscle and cognitive performance Distrofia muscular de Duchenne: imunoexpressão da alfa-distroglicana em musculatura esquelética e performance cognitiva |
| title_fullStr | Duchenne muscular dystrophy: alpha-dystroglycan immunoexpression in skeletal muscle and cognitive performance Distrofia muscular de Duchenne: imunoexpressão da alfa-distroglicana em musculatura esquelética e performance cognitiva |
| title_full_unstemmed | Duchenne muscular dystrophy: alpha-dystroglycan immunoexpression in skeletal muscle and cognitive performance Distrofia muscular de Duchenne: imunoexpressão da alfa-distroglicana em musculatura esquelética e performance cognitiva |
| title_short | Duchenne muscular dystrophy: alpha-dystroglycan immunoexpression in skeletal muscle and cognitive performance Distrofia muscular de Duchenne: imunoexpressão da alfa-distroglicana em musculatura esquelética e performance cognitiva |
| title_sort | duchenne muscular dystrophy alpha dystroglycan immunoexpression in skeletal muscle and cognitive performance distrofia muscular de duchenne imunoexpressao da alfa distroglicana em musculatura esqueletica e performance cognitiva |
| topic | distrofia muscular de Duchenne alfa-distroglicana déficit cognitivo distrofina Duchenne muscular dystrophy alpha-dystroglycan cognitive impairment dystrophin |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2005000600015 |
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