Editorial: Imaging of Neuromuscular Diseases

Díaz‐Manera, Jordi; Pichiecchio, Anna; Santini, Francesco; Filosto, Massimiliano

doi:10.3389/fneur.2021.814579

Cited by 3 publications

(4 citation statements)

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“…Und es ist ein Medikament entwickelt worden, das durch eine bessere Aufnahme des rekombinanten Enzyms eine höhere Effektivität hat“, erläutert Prof. Andreas Hahn, Gießen. Die weiterentwickelte ERT mit Avalglucosidase alfa hat im Vergleich zu Alglucosidase alfa einen etwa 15-mal höheren Anteil an Mannose-6-Phosphat(M6P)-Resten, wodurch sich die M6P-Rezeptoraffinität erhöht und eine verbesserte Enzymaufnahme in die Muskelzellen erreicht werden kann 2 . Die Phase-III-Studie COMET zeigte für Avalglucosidase alfa vs. Alglucosidase alfa eine Nichtunterlegenheit sowie klinisch bedeutsame Verbesserungen der Atem- und Muskelfunktion 2 .…”

Section: Morbus Pompe – Pädiater In Der Schlüsselposition Bei Frühdia...unclassified

“…Die weiterentwickelte ERT mit Avalglucosidase alfa hat im Vergleich zu Alglucosidase alfa einen etwa 15-mal höheren Anteil an Mannose-6-Phosphat(M6P)-Resten, wodurch sich die M6P-Rezeptoraffinität erhöht und eine verbesserte Enzymaufnahme in die Muskelzellen erreicht werden kann 2 . Die Phase-III-Studie COMET zeigte für Avalglucosidase alfa vs. Alglucosidase alfa eine Nichtunterlegenheit sowie klinisch bedeutsame Verbesserungen der Atem- und Muskelfunktion 2 . Die Mini-COMET-Studie untermauert zudem die positiven Auswirkungen von Avalglucosidase alfa bei IOPD-Patienten auf die motorischen und kardialen Funktionen 3 .…”

Section: Morbus Pompe – Pädiater In Der Schlüsselposition Bei Frühdia...unclassified

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Was tut sich bei Neuroinflammation und Neurometabolik?

Borchard-Tuch

2024

Kinder- und Jugendmedizin

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Die 48. Jahrestagung der Gesellschaft für Neuropädiatrie (GNP) im November in Dortmund verbindete Wissenschaft und Praxis und bietete eine breite Palette zur Weiterbildung. Einen Schwerpunkt bildeten die seltenen Erkrankungen. Zunehmende Kenntnisse ätiologischer Zusammenhänge und Entwicklungen neuer Therapieverfahren lassen auf mildere Krankheitsverläufe und eine bessere Langzeitprognose hoffen.

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Section: Morbus Pompe – Pädiater In Der Schlüsselposition Bei Frühdia...unclassified

Was tut sich bei Neuroinflammation und Neurometabolik?

Borchard-Tuch

2024

Kinder- und Jugendmedizin

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“…Recently, the research community has been increasingly interested in texture analysis as an alternative method for biomarker assessment in NMD. 26,27 However, texture analysis is still complex to be performed, analyzed, and interpreted.…”

Section: Introductionmentioning

confidence: 99%

“…Especially for the heterogeneously affected skeletal muscles that are typically present in various NMD, the routinely performed mean or median value analyses of MR biomarkers are questionable, and a deeper analysis for complex skewed distributions is necessary. Recently, the research community has been increasingly interested in texture analysis as an alternative method for biomarker assessment in NMD 26,27 . However, texture analysis is still complex to be performed, analyzed, and interpreted.…”

Section: Introductionmentioning

confidence: 99%

Beyond mean value analysis – a voxel‐based analysis of the quantitative MR biomarker water T₂ in the presence of fatty infiltration in skeletal muscle tissue of patients with neuromuscular diseases

et al. 2022

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The main pathologies in the muscles of patients with neuromuscular diseases (NMD) are fatty infiltration and edema. Recently, quantitative magnetic resonance (MR) imaging for determination of the MR biomarkers proton density fat fraction (PDFF) and water T2 (T2w) has been advanced. Biophysical effects or pathology can have different effects on MR biomarkers. Thus, for heterogeneously affected muscles, the routinely performed mean or median value analyses of MR biomarkers are questionable. Our work presents a voxel‐based histogram analysis of PDFF and T2w images to point out potential quantification errors. In 12 patients with NMD, chemical‐shift encoding‐based water‐fat imaging for PDFF and T2 mapping with spectral adiabatic inversion recovery (SPAIR) for T2w determination was performed. Segmentation of nine thigh muscles was performed bilaterally (n = 216). PDFF and T2 maps were coregistered. A voxel‐based comparison of PDFF and T2w showed a decreased T2w with increasing PDFF. Mean T2w and mean T2wwithout fatty voxels (PDFF < 10%) show good agreement, whereas standard deviation (σ) T2w and σ T2wwithout fatty voxels show increasing difference with increasing values of σ. Thereby two subgroups can be observed, referring to muscles in which the exclusion of fatty voxels has a negligible influence versus muscles in which a strong dependency of the T2w value distribution on the exclusion of fatty voxels is present. Because of the two opposite effects that influence T2w in a voxel, namely, (i) a pathophysiologically increased water mobility leading to T2w elevation, and (ii) a dependency of T2w on the PDFF leading to decreased T2w, the T2w distribution within a muscle might be heterogenous and the routine mean or median analysis can lead to a misinterpretation of the muscle health. It was concluded that muscle T2w mean values can wrongly suggest healthy muscle tissue. A deeper analysis of the underlying value distribution is necessary. Therefore, a quantitative analysis of T2w histograms is a potential alternative.

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Neuromuscular imaging in clinical practice: an ESNR survey of 30 centers

Gerevini,

Cristiano,

D’Anna

et al. 2023

Neuroradiology

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Editorial: Imaging of Neuromuscular Diseases

Cited by 3 publications

References 1 publication

Was tut sich bei Neuroinflammation und Neurometabolik?

Was tut sich bei Neuroinflammation und Neurometabolik?

Beyond mean value analysis – a voxel‐based analysis of the quantitative MR biomarker water T₂ in the presence of fatty infiltration in skeletal muscle tissue of patients with neuromuscular diseases

Neuromuscular imaging in clinical practice: an ESNR survey of 30 centers

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Editorial: Imaging of Neuromuscular Diseases

Cited by 3 publications

References 1 publication

Was tut sich bei Neuroinflammation und Neurometabolik?

Was tut sich bei Neuroinflammation und Neurometabolik?

Beyond mean value analysis – a voxel‐based analysis of the quantitative MR biomarker water T2 in the presence of fatty infiltration in skeletal muscle tissue of patients with neuromuscular diseases

Neuromuscular imaging in clinical practice: an ESNR survey of 30 centers

Contact Info

Product

Resources

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Beyond mean value analysis – a voxel‐based analysis of the quantitative MR biomarker water T₂ in the presence of fatty infiltration in skeletal muscle tissue of patients with neuromuscular diseases