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  • 1
    Online Resource
    Online Resource
    Whole-body muscle MRI of patients with MATR3-associated distal myopathy reveals a distinct pattern of muscular involvement and highlights the value of whole-body examination
    Springer Science and Business Media LLC ; 2020
    In:  Journal of Neurology Vol. 267, No. 8 ( 2020-08), p. 2408-2420
    Details
    In: Journal of Neurology, Springer Science and Business Media LLC, Vol. 267, No. 8 ( 2020-08), p. 2408-2420
    Abstract: MATR3-associated distal myopathy is a rare distal myopathy predominantly affecting lower legs as well as wrist- and finger extensors. Whilst most distal myopathies are clinically and genetically well characterized, diagnosis often remains challenging. Pattern-based magnetic resonance imaging (MRI) approaches offer valuable additional information. However, a consistent pattern of muscular affection is missing for most distal myopathies. Thus, the aim of the present study was to establish a disease-specific pattern of muscular involvement in MATR3-associated distal myopathy using whole-body MRI. Methods 15 patients (25–79 years of age, 7 female) with MATR3-associated distal myopathy were subjected to whole-body MRI. The grade of fatty involution for individual muscles was determined using Fischer-Grading. Results were compared to established MRI-patterns of other distal myopathies. Results There was a predominant affection of the distal lower extremities. Lower legs showed a severe fatty infiltration, prominently affecting gastrocnemius and soleus muscle. In thighs, a preferential involvement of semimembranous and biceps femoris muscle was observed. Severe affection of gluteus minimus muscle as well as axial musculature, mainly affecting the thoracic segments, was seen. A sufficient discrimination to other forms of distal myopathy based solely on MRI-findings of the lower extremities was not possible. However, the inclusion of additional body parts seemed to yield specificity. Interpretation Muscle MRI of patients with MATR3-associated distal myopathy revealed a distinct pattern of muscular involvement. The usage of whole-body muscle MRI provided valuable additional findings as compared to regular MRI of the lower extremities to improve distinction from other disease entities.
    Type of Medium: Online Resource
    ISSN: 0340-5354 , 1432-1459
    URL: Article
    DOI: 10.1007/s00415-020-09862-9
    RVK:
    XA 10000
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2020
    detail.hit.zdb_id: 1421299-7
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  • 2
    Online Resource
    Online Resource
    Quantitative whole-body muscle MRI in idiopathic inflammatory myopathies including polymyositis with mitochondrial pathology: indications for a disease spectrum
    Springer Science and Business Media LLC ; 2024
    In:  Journal of Neurology
    Details
    In: Journal of Neurology, Springer Science and Business Media LLC
    Abstract: Inflammatory myopathies (IIM) include dermatomyositis (DM), sporadic inclusion body myositis (sIBM), immune-mediated necrotizing myopathy (IMNM), and overlap myositis (OLM)/antisynthetase syndrome (ASyS). There is also a rare variant termed polymyositis with mitochondrial pathology (PM-Mito), which is considered a sIBM precursor. There is no information regarding muscle MRI for this rare entity. The aim of this study was to compare MRI findings in IIM, including PM-Mito. Methods This retrospective analysis included 41 patients (7 PM-Mito, 11 sIBM, 11 PM/ASyS/OLM, 12 IMNM) and 20 healthy controls. Pattern of muscle involvement was assessed by semiquantitative evaluation, while Dixon method was used to quantify muscular fat fraction. Results The sIBM typical pattern affecting the lower extremities was not found in the majority of PM-Mito-patients. Intramuscular edema in sIBM and PM-Mito was limited to the lower extremities, whereas IMNM and PM/ASyS/OLM showed additional edema in the trunk. Quantitative assessment showed increased fat content in sIBM, with an intramuscular proximo-distal gradient. Similar changes were also found in a few PM-Mito- and PM/ASyS/OLM patients. In sIBM and PM-Mito, mean fat fraction of several muscles correlated with clinical involvement. Interpretation As MRI findings in patients with PM-Mito relevantly differed from sIBM, the attribution of PM-Mito as sIBM precursor should be critically discussed. Some patients in PM/ASyS/OLM and PM-Mito group showed MR-morphologic features predominantly observed in sIBM, indicative of a spectrum from PM/ASyS/OLM toward sIBM. In some IIM subtypes, MRI may serve as a biomarker of disease severity.
    Type of Medium: Online Resource
    ISSN: 0340-5354 , 1432-1459
    URL: Article
    DOI: 10.1007/s00415-024-12191-w
    RVK:
    XA 10000
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2024
    detail.hit.zdb_id: 1421299-7
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  • 3
    Online Resource
    Online Resource
    Bildgebung der Muskulatur bei Neuromuskulären Erkrankungen – von der Initialdiagnostik bis zur Verlaufsbeurteilung
    Georg Thieme Verlag KG ; 2022
    In:  Klinische Neurophysiologie Vol. 53, No. 01 ( 2022-03), p. 36-47
    Details
    In: Klinische Neurophysiologie, Georg Thieme Verlag KG, Vol. 53, No. 01 ( 2022-03), p. 36-47
    Abstract: Die bildgebende Diagnostik hat sich zu einem integralen Element der Betreuung von PatientInnen mit neuromuskulären Erkrankungen entwickelt. Als wesentliches Diagnostikum ist hierbei die Magnetresonanztomografie als breit verfügbares und vergleichsweise standardisiertes Untersuchungsverfahren etabliert, wobei die Sonografie der Muskulatur bei hinreichend erfahrenem Untersucher ebenfalls geeignet ist, wertvolle diagnostische Informationen zu liefern. Das CT hingegen spielt eine untergeordnete Rolle und sollte nur bei Kontraindikationen für eine MRT in Erwägung gezogen werden. Zunächst wurde die Bildgebung bei Muskelerkrankungen primär in der Initialdiagnostik unter vielfältigen Fragestellungen eingesetzt. Das Aufkommen innovativer Therapiekonzepte bei verschiedenen neuromuskulären Erkrankungen machen neben einer möglichst frühzeitigen Diagnosestellung insbesondere auch eine multimodale Verlaufsbeurteilung zur Evaluation des Therapieansprechens notwendig. Auch hier wird die Bildgebung der Muskulatur als objektiver Parameter des Therapieerfolges intensiv diskutiert und in Forschung wie Praxis zunehmend verwendet.
    Type of Medium: Online Resource
    ISSN: 1434-0275 , 1439-4081
    URL: Article
    DOI: 10.1055/a-1738-5356
    Language: German
    Publisher: Georg Thieme Verlag KG
    Publication Date: 2022
    detail.hit.zdb_id: 2063780-9
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  • 4
    Online Resource
    Online Resource
    Update Distale Myopathien
    Georg Thieme Verlag KG ; 2022
    In:  Klinische Neurophysiologie Vol. 53, No. 01 ( 2022-03), p. 23-28
    Details
    In: Klinische Neurophysiologie, Georg Thieme Verlag KG, Vol. 53, No. 01 ( 2022-03), p. 23-28
    Abstract: Die Distalen Myopathien umfassen eine Gruppe von genetisch determinierten Muskelerkrankungen bei denen Paresen und eine fortschreitende Atrophie der distalen Muskelgruppen im Vordergrund stehen. Der klinische Phänotyp, der Erkrankungsbeginn, der Vererbungsmodus sowie histologische Veränderungen helfen die einzelnen Formen zu differenzieren. Das klinische und genetische Spektrum ist allerdings heterogen. In den letzten Jahren hat durch die erweiterte genetische Diagnostik die Anzahl der nachgewiesenen Mutationen exponentiell zugenommen. Im folgenden Beitrag werden die Klassifikation, die klinischen Besonderheiten und die relevanten genetischen Aspekte dargestellt.
    Type of Medium: Online Resource
    ISSN: 1434-0275 , 1439-4081
    URL: Article
    DOI: 10.1055/a-1737-8273
    Language: German
    Publisher: Georg Thieme Verlag KG
    Publication Date: 2022
    detail.hit.zdb_id: 2063780-9
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