دورية أكاديمية
أعرض في EDS

Whole-Body MRI in Limb Girdle Muscular Dystrophy Type R1/2A: Correlation With Clinical Scores.

التفاصيل البيبلوغرافية
العنوان: Whole-Body MRI in Limb Girdle Muscular Dystrophy Type R1/2A: Correlation With Clinical Scores.
المؤلفون: Aivazoglou LU; Department of Radiology and Diagnostic Imaging, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil.; Division of Musculoskeletal Imaging, Laboratório Delboni Auriemo / DASA (Diagnósticos da América SA), Barueri, SP, Brazil., Guimarães JB; Department of Radiology and Diagnostic Imaging, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil., Costa MAF; Department of Radiology and Diagnostic Imaging, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil.; Division of Musculoskeletal Imaging, Laboratório Delboni Auriemo / DASA (Diagnósticos da América SA), Barueri, SP, Brazil., Aihara AY; Department of Radiology and Diagnostic Imaging, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil.; Division of Musculoskeletal Imaging, Laboratório Delboni Auriemo / DASA (Diagnósticos da América SA), Barueri, SP, Brazil., Cardoso FN; Department of Radiology and Diagnostic Imaging, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil., Pinto WBVR; Division of Neuromuscular Diseases, Department of Neurology and Neurosurgery, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil., de Souza PVS; Division of Neuromuscular Diseases, Department of Neurology and Neurosurgery, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil., da Silva AMS; Departamento de Neurologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil., Zanoteli E; Departamento de Neurologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil., Oliveira ASB; Division of Neuromuscular Diseases, Department of Neurology and Neurosurgery, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil., Carvalho AAS; Laboratório de Doenças Neuromusculares da Faculdade de Medicina do ABC, Departamento de Neurociências, Santo André, SP, Brazil., Fernandes ADRC; Department of Radiology and Diagnostic Imaging, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil.
المصدر: Muscle & nerve [Muscle Nerve] 2022 Oct; Vol. 66 (4), pp. 471-478. Date of Electronic Publication: 2022 Aug 10.
نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't
اللغة: English
بيانات الدورية: Publisher: John Wiley & Sons Country of Publication: United States NLM ID: 7803146 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1097-4598 (Electronic) Linking ISSN: 0148639X NLM ISO Abbreviation: Muscle Nerve Subsets: MEDLINE
أسماء مطبوعة: Publication: <2005-> : Hoboken, NJ : John Wiley & Sons
Original Publication: New York, NY : John Wiley & Sons
مواضيع طبية MeSH: Muscular Diseases* , Muscular Dystrophies, Limb-Girdle*, Humans ; Magnetic Resonance Imaging/methods ; Muscle, Skeletal/diagnostic imaging
مستخلص: Introduction/aim: The most common limb girdle muscular dystrophy (LGMD) worldwide is LGMD type R1 (LGMDR1). The aim of this study was to correlate the MRI findings with functional scores and to describe the whole-body MRI (WBMRI) pattern in a LGMDR1 Brazilian cohort.
Methods: LGMDR1 patients under follow-up in three centers were referred for the study. Clinical data were collected and a functional evaluation was performed, consisting of Gardner-Medwin and Walton (GMW) and Brooke scales. All patients underwent a WBMRI study (1.5T) with axial T1 and STIR images. Fifty-one muscles were semiquantitatively assessed regarding fatty infiltration and muscle edema.
Results: The study group consisted of 18 patients. The highest fatty infiltration scores involved the serratus anterior, biceps femoris long head, adductor magnus, and lumbar erector spinae. There was a latero-medial and caudo-cranial descending gradient of involvement of the paravertebral muscles, with erector spinae being significantly more affected than the transversospinalis muscles (p < 0.05). A striped appearance that has been dubbed the "pseudocollagen sign" was present in 72% of the patients. There was a positive correlation between the MRI score and GMW (Rho:0.83) and Brooke (Rho:0.53) scores.
Discussion: WBMRI in LGMDR1 allows a global patient evaluation including involvement of the paraspinal muscles, usually an underestimated feature in the clinical and imaging study of myopathies. Knowledge of the WBMRI pattern of LGMDR1 involvement can be useful in the diagnostic approach and in future studies to identify the best target muscles to serve as outcome measures in clinical trials.
(© 2022 Wiley Periodicals LLC.)
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فهرسة مساهمة: Keywords: CAPN3; LGMD; MRI; limb-girdle muscular dystrophy; muscle dystrophy
SCR Disease Name: Limb-girdle muscular dystrophy type 2A
تواريخ الأحداث: Date Created: 20220727 Date Completed: 20220920 Latest Revision: 20221024
رمز التحديث: 20221213
DOI: 10.1002/mus.27686
PMID: 35894554
قاعدة البيانات: MEDLINE
الوصف
تدمد:1097-4598
DOI:10.1002/mus.27686