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Inclusion body myositis with early onset: a population-based study.

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Title: Inclusion body myositis with early onset: a population-based study.
Authors: Lindgren, Ulrika1,2 (AUTHOR) ulrika.lindgren@gu.se, Hedberg-Oldfors, Carola1 (AUTHOR), Pullerits, Rille3,4 (AUTHOR), Lindberg, Christopher2 (AUTHOR), Oldfors, Anders1 (AUTHOR)
Source: Journal of Neurology. Nov2023, Vol. 270 Issue 11, p5483-5492. 10p.
Subject Terms: *INCLUSION body myositis, *CYTOCHROME oxidase, *MITOCHONDRIAL DNA, *MUSCLE weakness, *NEMALINE myopathy, *MUSCLE strength
Geographic Terms: SWEDEN
Abstract: Introduction: Inclusion body myositis (IBM), an inflammatory myopathy with progressive weakness without efficient treatment, typically presents after 45 years of age and younger patients are sparsely studied. Methods: In a population-based study during a 33-year period, 142 patients with IBM were identified in western Sweden. Six patients fell outside the European Neuromuscular Centre 2011 criteria for IBM due to young age at symptom onset, verified by a muscle biopsy < 50 years of age. These were defined as early-onset IBM and included in this study. Medical records, muscle strength, comorbidities, muscle biopsies, and nuclear- and mitochondrial DNA were examined and compared with patients with IBM and age matched controls from the same population. Results: The median age at symptom onset was 36 (range 34–45) years and at diagnosis 43 (range 38–58) years. Four patients were deceased at a median age of 59 (range 50–75) years. The median survival from diagnosis was 14 (range 10–18) years. The prevalence December 31 2017 was 1.2 per million inhabitants and the mean incidence 0.12 patients per million inhabitants and year. The mean decline in quadriceps strength ± 1 standard deviation was 1.21 ± 0.2 Newton or 0.91 ± 0.2% per month and correlated to time from diagnosis (p < 0.001). Five patients had swallowing difficulties. All patients displayed mitochondrial changes in muscle including cytochrome c oxidase deficiency and the mitochondrial DNA mutation load was high. Conclusions: Early-onset IBM is a severe disease, causing progressive muscle weakness, high muscle mitochondrial DNA mutation load and a reduced cumulative survival in young and middle-aged individuals. [ABSTRACT FROM AUTHOR]
Copyright of Journal of Neurology is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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  Data: Inclusion body myositis with early onset: a population-based study.
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  Data: &lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Lindgren%2C+Ulrika%22&quot;&gt;Lindgren, Ulrika&lt;/searchLink&gt;&lt;relatesTo&gt;1,2&lt;/relatesTo&gt; (AUTHOR)&lt;i&gt; ulrika.lindgren@gu.se&lt;/i&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Hedberg-Oldfors%2C+Carola%22&quot;&gt;Hedberg-Oldfors, Carola&lt;/searchLink&gt;&lt;relatesTo&gt;1&lt;/relatesTo&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Pullerits%2C+Rille%22&quot;&gt;Pullerits, Rille&lt;/searchLink&gt;&lt;relatesTo&gt;3,4&lt;/relatesTo&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Lindberg%2C+Christopher%22&quot;&gt;Lindberg, Christopher&lt;/searchLink&gt;&lt;relatesTo&gt;2&lt;/relatesTo&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Oldfors%2C+Anders%22&quot;&gt;Oldfors, Anders&lt;/searchLink&gt;&lt;relatesTo&gt;1&lt;/relatesTo&gt; (AUTHOR)
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  Data: &lt;searchLink fieldCode=&quot;JN&quot; term=&quot;%22Journal+of+Neurology%22&quot;&gt;Journal of Neurology&lt;/searchLink&gt;. Nov2023, Vol. 270 Issue 11, p5483-5492. 10p.
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– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: Introduction: Inclusion body myositis (IBM), an inflammatory myopathy with progressive weakness without efficient treatment, typically presents after 45 years of age and younger patients are sparsely studied. Methods: In a population-based study during a 33-year period, 142 patients with IBM were identified in western Sweden. Six patients fell outside the European Neuromuscular Centre 2011 criteria for IBM due to young age at symptom onset, verified by a muscle biopsy &lt; 50 years of age. These were defined as early-onset IBM and included in this study. Medical records, muscle strength, comorbidities, muscle biopsies, and nuclear- and mitochondrial DNA were examined and compared with patients with IBM and age matched controls from the same population. Results: The median age at symptom onset was 36 (range 34–45) years and at diagnosis 43 (range 38–58) years. Four patients were deceased at a median age of 59 (range 50–75) years. The median survival from diagnosis was 14 (range 10–18) years. The prevalence December 31 2017 was 1.2 per million inhabitants and the mean incidence 0.12 patients per million inhabitants and year. The mean decline in quadriceps strength &#177; 1 standard deviation was 1.21 &#177; 0.2 Newton or 0.91 &#177; 0.2% per month and correlated to time from diagnosis (p &lt; 0.001). Five patients had swallowing difficulties. All patients displayed mitochondrial changes in muscle including cytochrome c oxidase deficiency and the mitochondrial DNA mutation load was high. Conclusions: Early-onset IBM is a severe disease, causing progressive muscle weakness, high muscle mitochondrial DNA mutation load and a reduced cumulative survival in young and middle-aged individuals. [ABSTRACT FROM AUTHOR]
– Name: AbstractSuppliedCopyright
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  Data: &lt;i&gt;Copyright of Journal of Neurology is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder&#39;s express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.&lt;/i&gt; (Copyright applies to all Abstracts.)
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        Value: 10.1007/s00415-023-11878-w
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        Text: English
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        Type: general
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      – SubjectFull: CYTOCHROME oxidase
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              Text: Nov2023
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