Spinal Muscular Atrophy
Spinal muscular atrophy (SMA) is among the commonest degenerative disorders of the nervous system in childhood. This is an inherited autosomal recessive disease which results in premature death of anterior horn cells of the spinal cord and is manifested by progressive weakness and atrophy of skeleta...
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S. Karger AG
1999
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Online Access: | http://dx.doi.org/10.1159/000026221 https://www.karger.com/Article/Pdf/26221 |
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crskarger:10.1159/000026221 2024-10-13T14:08:22+00:00 Spinal Muscular Atrophy Ludvigsson, Petur Olafsson, Elias Hauser, W. Allen 1999 http://dx.doi.org/10.1159/000026221 https://www.karger.com/Article/Pdf/26221 en eng S. Karger AG https://www.karger.com/Services/SiteLicenses https://www.karger.com/Services/SiteLicenses Neuroepidemiology volume 18, issue 5, page 265-269 ISSN 0251-5350 1423-0208 journal-article 1999 crskarger https://doi.org/10.1159/000026221 2024-09-18T04:06:35Z Spinal muscular atrophy (SMA) is among the commonest degenerative disorders of the nervous system in childhood. This is an inherited autosomal recessive disease which results in premature death of anterior horn cells of the spinal cord and is manifested by progressive weakness and atrophy of skeletal muscles. Few studies have looked at the frequency of the disease in a defined population. We identified all patients diagnosed with SMA in Iceland during a 15-year period. The diagnosis is based on typical symptoms and supported by results of electromyography/nerve conduction studies and muscle biopsy. The average annual incidence was 13.7 per 100,000 live births for all types of SMA, which is similar to that reported in other population-based studies. Article in Journal/Newspaper Iceland Karger Neuroepidemiology 18 5 265 269 |
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Karger |
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English |
description |
Spinal muscular atrophy (SMA) is among the commonest degenerative disorders of the nervous system in childhood. This is an inherited autosomal recessive disease which results in premature death of anterior horn cells of the spinal cord and is manifested by progressive weakness and atrophy of skeletal muscles. Few studies have looked at the frequency of the disease in a defined population. We identified all patients diagnosed with SMA in Iceland during a 15-year period. The diagnosis is based on typical symptoms and supported by results of electromyography/nerve conduction studies and muscle biopsy. The average annual incidence was 13.7 per 100,000 live births for all types of SMA, which is similar to that reported in other population-based studies. |
format |
Article in Journal/Newspaper |
author |
Ludvigsson, Petur Olafsson, Elias Hauser, W. Allen |
spellingShingle |
Ludvigsson, Petur Olafsson, Elias Hauser, W. Allen Spinal Muscular Atrophy |
author_facet |
Ludvigsson, Petur Olafsson, Elias Hauser, W. Allen |
author_sort |
Ludvigsson, Petur |
title |
Spinal Muscular Atrophy |
title_short |
Spinal Muscular Atrophy |
title_full |
Spinal Muscular Atrophy |
title_fullStr |
Spinal Muscular Atrophy |
title_full_unstemmed |
Spinal Muscular Atrophy |
title_sort |
spinal muscular atrophy |
publisher |
S. Karger AG |
publishDate |
1999 |
url |
http://dx.doi.org/10.1159/000026221 https://www.karger.com/Article/Pdf/26221 |
genre |
Iceland |
genre_facet |
Iceland |
op_source |
Neuroepidemiology volume 18, issue 5, page 265-269 ISSN 0251-5350 1423-0208 |
op_rights |
https://www.karger.com/Services/SiteLicenses https://www.karger.com/Services/SiteLicenses |
op_doi |
https://doi.org/10.1159/000026221 |
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Neuroepidemiology |
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18 |
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5 |
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265 |
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269 |
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1812815062071508992 |