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Muscle diffusion tensor imaging in glycogen storage disease V (McArdle disease).

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Title: Muscle diffusion tensor imaging in glycogen storage disease V (McArdle disease).
Authors: Rehmann, R.1, Schlaffke, L.1,2 lara.schlaffke@ruhr-uni-bochum.de, Froeling, M.2, Kley, R. A.1, Kühnle, E.1, De Marées, M.3, Forsting, J.1, Rohm, M.1, Tegenthoff, M.1, Schmidt-Wilcke, T.4,5, Vorgerd, M.1
Source: European Radiology. Jun2019, Vol. 29 Issue 6, p3224-3232. 9p. 3 Color Photographs, 4 Charts, 1 Graph.
Subjects: Glycogen storage disease, Diffusion tensor imaging, Rectus femoris muscles, Thigh, Biceps femoris, Vastus lateralis, Skeletal muscle
Abstract: Purpose: To evaluate differences in diffusion parameters in thigh muscles in patients with glycogen storage disease type V (McArdle disease) using muscle diffusion tensor imaging (mDTI) compared to healthy controls METHODS: In this prospective study, we evaluated thigh muscles from hip to knee of 10 McArdle patients (5 female, mean age 33.7 ± 14.4 years) and 10 healthy age- and gender-matched volunteers. MRI scans were performed at 3 T and comprised mDTI, T1-weighted and T2-weighted imaging between May 2015 and May 2017. Needle biopsy of the vastus lateralis muscle was performed in three McArdle patients. The muscle tissue was analyzed by using histochemical and enzyme-histochemical techniques for glycogen content and histopathological changes. Mean values of the eigenvalues (λ1-λ3), fractional anisotropy (FA), and mean diffusivity (MD) were obtained for the vastus lateralis, vastus medialis, rectus femoris, biceps femoris, semitendinosus, and semimembranosus and compared between groups using Student's t tests, as well as ANCOVA; significance level was set at p < 0.05.Results: Needle biopsy showed intracellular glycogen accumulation in skeletal muscle fibers of three McArdle patients. Extracellular histopathological changes were not found. Muscle DTI analysis did not show statistically significant differences between patients and controls for any of the muscles.Conclusion: Despite intracellular glycogen accumulation in the three biopsy samples, mDTI parameters were not altered in McArdle patients compared to controls. We conclude that the currently used mDTI acquisition and processing lack the sensitivity to detect intracellular changes due to accumulated glycogen in this cohort of McArdle patients.Key Points: • Despite intracellular glycogen accumulation in three examined biopsy samples, mDTI parameters were not altered in McArdle patients compared to controls. • In its current form, diffusion MR does not provide additional information in quantifying intracellular glycogen accumulations within skeletal muscle fibers in McArdle patients. [ABSTRACT FROM AUTHOR]
Copyright of European Radiology is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. 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: Muscle diffusion tensor imaging in glycogen storage disease V (McArdle disease).
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  Data: &lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Rehmann%2C+R%2E%22&quot;&gt;Rehmann, R.&lt;/searchLink&gt;&lt;relatesTo&gt;1&lt;/relatesTo&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Schlaffke%2C+L%2E%22&quot;&gt;Schlaffke, L.&lt;/searchLink&gt;&lt;relatesTo&gt;1,2&lt;/relatesTo&gt;&lt;i&gt; lara.schlaffke@ruhr-uni-bochum.de&lt;/i&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Froeling%2C+M%2E%22&quot;&gt;Froeling, M.&lt;/searchLink&gt;&lt;relatesTo&gt;2&lt;/relatesTo&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Kley%2C+R%2E+A%2E%22&quot;&gt;Kley, R. A.&lt;/searchLink&gt;&lt;relatesTo&gt;1&lt;/relatesTo&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22K&#252;hnle%2C+E%2E%22&quot;&gt;K&#252;hnle, E.&lt;/searchLink&gt;&lt;relatesTo&gt;1&lt;/relatesTo&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22De+Mar&#233;es%2C+M%2E%22&quot;&gt;De Mar&#233;es, M.&lt;/searchLink&gt;&lt;relatesTo&gt;3&lt;/relatesTo&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Forsting%2C+J%2E%22&quot;&gt;Forsting, J.&lt;/searchLink&gt;&lt;relatesTo&gt;1&lt;/relatesTo&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Rohm%2C+M%2E%22&quot;&gt;Rohm, M.&lt;/searchLink&gt;&lt;relatesTo&gt;1&lt;/relatesTo&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Tegenthoff%2C+M%2E%22&quot;&gt;Tegenthoff, M.&lt;/searchLink&gt;&lt;relatesTo&gt;1&lt;/relatesTo&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Schmidt-Wilcke%2C+T%2E%22&quot;&gt;Schmidt-Wilcke, T.&lt;/searchLink&gt;&lt;relatesTo&gt;4,5&lt;/relatesTo&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Vorgerd%2C+M%2E%22&quot;&gt;Vorgerd, M.&lt;/searchLink&gt;&lt;relatesTo&gt;1&lt;/relatesTo&gt;
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  Data: &lt;searchLink fieldCode=&quot;JN&quot; term=&quot;%22European+Radiology%22&quot;&gt;European Radiology&lt;/searchLink&gt;. Jun2019, Vol. 29 Issue 6, p3224-3232. 9p. 3 Color Photographs, 4 Charts, 1 Graph.
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  Data: &lt;bold&gt;Purpose: &lt;/bold&gt;To evaluate differences in diffusion parameters in thigh muscles in patients with glycogen storage disease type V (McArdle disease) using muscle diffusion tensor imaging (mDTI) compared to healthy controls METHODS: In this prospective study, we evaluated thigh muscles from hip to knee of 10 McArdle patients (5 female, mean age 33.7 &#177; 14.4&#160;years) and 10 healthy age- and gender-matched volunteers. MRI scans were performed at 3&#160;T and comprised mDTI, T1-weighted and T2-weighted imaging between May 2015 and May 2017. Needle biopsy of the vastus lateralis muscle was performed in three McArdle patients. The muscle tissue was analyzed by using histochemical and enzyme-histochemical techniques for glycogen content and histopathological changes. Mean values of the eigenvalues (λ1-λ3), fractional anisotropy (FA), and mean diffusivity (MD) were obtained for the vastus lateralis, vastus medialis, rectus femoris, biceps femoris, semitendinosus, and semimembranosus and compared between groups using Student&#39;s t tests, as well as ANCOVA; significance level was set at p &lt; 0.05.&lt;bold&gt;Results: &lt;/bold&gt;Needle biopsy showed intracellular glycogen accumulation in skeletal muscle fibers of three McArdle patients. Extracellular histopathological changes were not found. Muscle DTI analysis did not show statistically significant differences between patients and controls for any of the muscles.&lt;bold&gt;Conclusion: &lt;/bold&gt;Despite intracellular glycogen accumulation in the three biopsy samples, mDTI parameters were not altered in McArdle patients compared to controls. We conclude that the currently used mDTI acquisition and processing lack the sensitivity to detect intracellular changes due to accumulated glycogen in this cohort of McArdle patients.&lt;bold&gt;Key Points: &lt;/bold&gt;• Despite intracellular glycogen accumulation in three examined biopsy samples, mDTI parameters were not altered in McArdle patients compared to controls. • In its current form, diffusion MR does not provide additional information in quantifying intracellular glycogen accumulations within skeletal muscle fibers in McArdle patients. [ABSTRACT FROM AUTHOR]
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  Data: &lt;i&gt;Copyright of European Radiology is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder&#39;s express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. 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/s00330-018-5885-1
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        Text: English
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      – SubjectFull: Glycogen storage disease
        Type: general
      – SubjectFull: Diffusion tensor imaging
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      – SubjectFull: Rectus femoris muscles
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