To date, Becker Muscular Dystrophy (BMD) and Inclusion Body Myositis (IBM) have no effective treatment yet; however, in the last few years, different and promising therapeutic strategies have been developed. MR advanced techniques (aMR) give an added value compared to conventional imaging and clinical assessment in the diagnosis of muscular pathologies, allowing to obtain a targeted early diagnosis based on specific biomarkers, to do the follow-up of the pathology and to assess the effectiveness of the treatments performed. The aim of this study is to validate the effectiveness of the quantitative Magnetic Resonance (qMR) as a method to objectively evaluate the clinical outcome by performing a correlation between qMR and the clinical and myometric measures in subjects with BMD and IBM. Twenty-one patients, aged between 19 and 80 years (56,4 ± 20,4), have undergone a qMR (1.3T); 11 subjects were affected by IBM, 9 by BMD. The acquisition of the MR images of lower limbs (thighs and legs) was performed for each of the subjects: the protocol was composed by two sequences of conventional imaging and three sequences of aMR: 1) Dixon Sequence to estimate the fat and water fraction (Fat Fraction, FF); 2) Multi - Echo Gradient - Echo Sequence to evaluate the relaxation T2 of the tissues (Relaxometry T2); 3) Diffusion Tensor Imaging (DTI) to assess the damage and the lipid infiltration in the muscles. The parameters of each of the sequences have been studied for both thighs and legs and the correlation between those parameters and clinical outputs has been performed. The mean basal value of FF for the thighs is 44,1 ± 10.1% (IBM) and 42 ± 15% (BMD), 32 ± 15% (IBM) and 23,6 ± 9.3% (BMD) for the legs. T2 mean value is 110,4 ± 12 (IBM) and 90,8 ± 10,12 (BMD) for the thighs, 86,4 ± 6,8 (IBM) and 73 ± 5,7 (BMD) for the legs. While there are no specific MRI studies for BMD subjects yet, the MRI analysis performed on IBM subjects confirms the results contained in literature. The statistical analysis shows strong results for BMD patients: significant and coherent correlations have been found between FF and T2 and all the clinical scores.
Ad oggi non esistono trattamenti terapeutici efficaci per la distrofia muscolare di Becker (BMD) e la miosite da corpi inclusi (IBM). Negli ultimi anni, però, sono state sviluppate strategie terapeutiche promettenti. Le tecniche avanzate di RM (aRM) forniscono un valore aggiunto rispetto all’imaging convenzionale e alla valutazione clinica nella diagnosi di patologie muscolari, permettendo una diagnosi precoce mirata su specifici biomarker diagnostici, consentendo il follow-up della patologia e permettendo di valutare l’efficacia dei trattamenti impiegati. Obiettivo di questo studio è convalidare l’efficacia della Risonanza Magnetica quantitativa (qRM) come metodo di valutazione oggettiva dell’outcome clinico effettuando una correlazione tra le misure di qRM e le valutazioni cliniche e miometriche nei pazienti affetti da BMD e IBM. Ventuno pazienti di età compresa tra 19 e 80 anni (56,4 ± 20,4) sono stati sottoposti a qRM mediante una RM da 1.5T; 11 affetti da IBM e 9 da BMD. Per ciascuno di essi sono state acquisite le immagini di RM di cosce e gambe. Il protocollo è composto da due sequenze di imaging convenzionale e tre sequenze aRM: 1) Sequenza Dixon per stima della frazione di acqua e grasso (Fat Fraction, FF); 2) Sequenza Gradient-Echo multi-echo per valutazione della rilassività T2 dei tessuti (Rilassometria T2); 3) Diffusion Tensor Imaging (DTI) per valutazione del danno e dell’infiltrazione lipidica a livello muscolare. I parametri di ognuna delle sequenze sono stati studiati a livello delle cosce e delle gambe ed è stata effettuata una correlazione tra i suddetti parametri e gli score clinici. Il valore basale medio di FF per le cosce è 44,1 ± 10.1% (IBM) e 42 ± 15% (BMD), mentre per le gambe è 32 ± 15% (IBM) e 23,6 ± 9.3% (BMD). Il valore medio di T2 per le cosce è 110,4 ± 12 (IBM) e 90,8 ± 10,12 (BMD), per le gambe è 86,4 ± 6,8 (IBM) e 73 ± 5,7 (BMD). Mentre per i BMD non esistono ancora studi specifici di MRI, l’analisi MRI sugli IBM conferma i risultati ottenuti in letteratura. Dall’analisi statistica i risultati migliori sono stati ottenuti per i BMD: si sono trovate correlazioni significative e coerenti tra FF e T2 e tutti gli score clinici.
Quantificazione mediante risonanza magnetica nucleare dell'infiltrazione lipidica e della sofferenza muscolare in miopatie degenerative e genetiche
MAZZI, FEDERICA
2016/2017
Abstract
To date, Becker Muscular Dystrophy (BMD) and Inclusion Body Myositis (IBM) have no effective treatment yet; however, in the last few years, different and promising therapeutic strategies have been developed. MR advanced techniques (aMR) give an added value compared to conventional imaging and clinical assessment in the diagnosis of muscular pathologies, allowing to obtain a targeted early diagnosis based on specific biomarkers, to do the follow-up of the pathology and to assess the effectiveness of the treatments performed. The aim of this study is to validate the effectiveness of the quantitative Magnetic Resonance (qMR) as a method to objectively evaluate the clinical outcome by performing a correlation between qMR and the clinical and myometric measures in subjects with BMD and IBM. Twenty-one patients, aged between 19 and 80 years (56,4 ± 20,4), have undergone a qMR (1.3T); 11 subjects were affected by IBM, 9 by BMD. The acquisition of the MR images of lower limbs (thighs and legs) was performed for each of the subjects: the protocol was composed by two sequences of conventional imaging and three sequences of aMR: 1) Dixon Sequence to estimate the fat and water fraction (Fat Fraction, FF); 2) Multi - Echo Gradient - Echo Sequence to evaluate the relaxation T2 of the tissues (Relaxometry T2); 3) Diffusion Tensor Imaging (DTI) to assess the damage and the lipid infiltration in the muscles. The parameters of each of the sequences have been studied for both thighs and legs and the correlation between those parameters and clinical outputs has been performed. The mean basal value of FF for the thighs is 44,1 ± 10.1% (IBM) and 42 ± 15% (BMD), 32 ± 15% (IBM) and 23,6 ± 9.3% (BMD) for the legs. T2 mean value is 110,4 ± 12 (IBM) and 90,8 ± 10,12 (BMD) for the thighs, 86,4 ± 6,8 (IBM) and 73 ± 5,7 (BMD) for the legs. While there are no specific MRI studies for BMD subjects yet, the MRI analysis performed on IBM subjects confirms the results contained in literature. The statistical analysis shows strong results for BMD patients: significant and coherent correlations have been found between FF and T2 and all the clinical scores.File | Dimensione | Formato | |
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https://hdl.handle.net/10589/135448