Magnetic resonance imaging of muscle in amyotrophic lateral sclerosis

Bryan, Wilson W.; Reisch, Joan; McDonald, George G.; Herbelin, Laura; Barohn, Richard J.; Fleckenstein, James L.

doi:10.1212/wnl.51.1.110

Cited by 55 publications

(49 citation statements)

References 9 publications

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“…Applications for measuring residual muscle mass in ALS patients by MRI and BIA have been previously reported. 8,22 In particular, BIA is reported to be safe, portable, highly reliable and relatively inexpensive. 23 However, both methods can measure only a limited segment of body muscles.…”

Section: Discussionmentioning

confidence: 99%

Creatinine/cystatin C ratio as a surrogate marker of residual muscle mass in amyotrophic lateral sclerosis

Tetsuka

Morita

Ikeguchi

et al. 2013

Neurology & Clinical Neurosc

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Aim: Identification of sensitive surrogate markers that indicate disease progression in amyotrophic lateral sclerosis might be useful in clinical trials and clinical care. Determination of the creatinine/cystatin C (Cr/CysC) ratio eliminates the effect of renal function on serum creatinine levels; therefore, we considered that the ratio might serve as a surrogate marker of residual muscle mass. We studied the Cr/ CysC ratio as a useful surrogate marker of residual muscle mass in patients with amyotrophic lateral sclerosis. Methods: A total of 103 participants were recruited: 62 patients with amyotrophic lateral sclerosis and 41 healthy controls. Serum levels of Cr and CysC were measured in both groups. We subsequently investigated the correlation between the Cr/CysC ratio and disease severity in patients with amyotrophic lateral sclerosis. Results:The ratio was significantly lower in the amyotrophic lateral sclerosis group than in the control group. Furthermore, the ratio decreased as the severity of amyotrophic lateral sclerosis increased. The Cr/CysC ratio might be a better and more reliable method than the serum Cr level as a means of monitoring residual muscle mass of the entire body in patients with amyotrophic lateral sclerosis. Conclusion:The present results show that the Cr/CysC ratio might be a suitable candidate for a useful and quantitative surrogate marker for the assessment of disease severity and progression in patients with amyotrophic lateral sclerosis.

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Section: Discussionmentioning

confidence: 99%

Creatinine/cystatin C ratio as a surrogate marker of residual muscle mass in amyotrophic lateral sclerosis

Tetsuka

Morita

Ikeguchi

et al. 2013

Neurology & Clinical Neurosc

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“…The significant gene expression changes resulting from both approaches were pooled in a unique data set, which was submitted to functional enrichment analysis using the DAVID bioinformatics resources 6.7, freely available from the National Institute of Allergy and Infectious Diseases at http://david.abcc.ncifcrf.gov [16,17] . To gain 2 Time from onset of symptoms to biopsy. 3 Ordinal scale of 12 items (max.…”

Section: Discussionmentioning

confidence: 99%

“…However, denervation-related changes on muscle histology are difficult to quantify, and their characterization depends on the objectivity of the neuropathologist. In addition, measures based on radiological techniques -such as echography or MRI [2,3] and electrical impedance myography [4] -have not shown enough sensitivity. Consequently, the lack of markers of disease progression is detrimental not only to the follow-up of patients in clinical practice, but also to the interpretation of the effects of potential disease-modifying drugs in therapeutic trials [5,6] .…”

Section: Introductionmentioning

confidence: 99%

Muscle Gene Expression Is a Marker of Amyotrophic Lateral Sclerosis Severity

et al. 2011

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Background: Amyotrophic lateral sclerosis (ALS) is a fatal adult-onset degenerative disease characterized by the loss of upper and lower motor neurons leading to progressive muscle atrophy and paralysis. The lack of molecular markers of the progression of disease is detrimental to clinical practice and therapeutic trials. Objective: This study was designed to identify gene expression changes in skeletal muscle that could reliably define the degree of disease severity. Methods: Gene expression profiles were obtained from the deltoid muscles of ALS patients and healthy subjects. Changes in differentially expressed genes were compared to the status of deltoid muscle disability, as determined by manual muscle testing, electrophysiology and the degree of myofiber atrophy. Functionally related genes were grouped by annotation analysis, and deltoid muscle injury was predicted using binary tree classifiers. Results: Two sets of 25 and 70 transcripts appeared differentially regulated exclusively in early and advanced states of deltoid muscle impairment, respectively. The expression of another set of 198 transcripts correlated with a composite score of muscle injury combining manual muscle testing and histological examination. From the totality of these expression changes, 155 transcripts distinguished advanced from early deltoid muscle impairment with 80% sensitivity and 100% specificity. Nine of these transcripts, known also to be regulated in ALS mouse and surgically denervated muscle, predicted the advanced disease status with 100% sensitivity and specificity. Conclusion: We provide robust gene expression changes that can be of practical use when monitoring ALS status and the effects of disease-modifying drugs.

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“…Chez les patients atteints de sclérose latérale amyotrophique, une augmentation significative du T2 global des muscles des jambes a été notée au terme d'une période d'observation de 4 mois, signant la progression des dégénérescences graisseuses. Le déclin de la contraction isométrique volontaire maximale lors d'une dorsiflexion du pied était corrélée à celle-ci (Bryan et al 1998). Alors que le contenu total en graisse des muscles de la jambe n'était pas affecté, les diabétiques de type 2 présentaient une distribution préférentielle des lipides en intramusculaire .…”

Section: Les Altérations Dégénératives Chroniquesunclassified

Imagerie et spectroscopie par résonance magnétique nucléaire du muscle strié squelettique

et al. 2016

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Au cours des dernières années, les traitements de nombreuses maladies neuromusculaires, jusqu'ici incurables, ont bénéficié d'importants progrès. Ce bouleversement contextuel a eu pour conséquence de stimuler le développement de nouveaux outils d'évaluation atraumatiques. Ceux-ci peuvent être classés en trois grandes catégories : les explorations fonctionnelles musculaires, les marqueurs des fluides biologiques et l'imagerie musculaire. Au sein de cette dernière, l'imagerie par résonance magnétique nucléaire (IRMN) offre un très large éventail de possibilités pour caractériser la composition, la fonction et le métabolisme du muscle strié squelettique. Aujourd'hui, trois indicateurs RMN sont couramment intégrés dans les protocoles de recherche clinique : 1) le volume musculaire ou l'aire d'une section musculaire transversale ciblée, 2) le pourcentage de graisse intramusculaire et 3) le T2 de l'eau musculaire. Ils permettent de quantifier respectivement la trophicité du muscle, les dégénérescences graisseuses chroniques et l'oedème tissulaire (ou plus généralement « l'activité de la maladie »). Un quatrième indicateur, le volume de tissu contractile est facilement dérivable des deux premiers. Les cartographies de fraction graisseuse, souvent issues de séquences Dixon, ont fait la preuve de leur utilité pour détecter de subtils changements de composition musculaire et se sont, à plusieurs reprises, révélées plus sensibles que les évaluations fonctionnelles standards. Cet indicateur sera probablement le premier parmi ceux proposés à être validé comme paramètre principal par les organismes de régulation. La diversité des contrastes obtenus par RMN permet d'explorer de nombreuses autres pistes de caractérisation du muscle squelettique et de nouveaux biomarqueurs RMN sont à attendre dans un avenir plus ou moins proche. Des séquences à TE ultra-courts (UTE), le rehaussement tardif post-injection de gadolinium et l'élastographie par RMN sont en cours d'étude pour l'évaluation de la fibrose interstitielle du muscle squelettique. De nombreuses options existent pour mesurer la perfusion et l'oxygénation du muscle par RMN. La RMN de diffusion ainsi que l'utilisation d'algorithmes d'analyse de texture pourraient apporter des informations supplémentaires sur l'organisation musculaire aux échelles respectivement microscopiques et mésoscopiques. La spectroscopie RMN du phosphore 31 P est la technique de référence pour l'évaluation atraumatique de l'énergétique musculaire pendant et après exercice. Le spectre 31 P du muscle dystrophique au repos est notablement altéré, et plusieurs de ses résonances informent sur l'intégrité de la membrane cellulaire. D'importants efforts sont consacrés à l'accélération de l'acquisition des images au travers plusieurs approches, allant de l'extraction du contenu en graisse et des cartographies T2 au départ d'une unique séquence, jusqu'à l'utilisation de scénarios d'acquisition partielle des matrices. Dans un avenir proche, une diminution spectaculaire du temps d'acquisition est attendue. Cela ren...

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Magnetic resonance imaging of muscle in amyotrophic lateral sclerosis

Abstract: Of the MRI characteristics studied, T2 relaxation time was the best indicator of motor neuron dysfunction and may have a role in objective evaluation of motor neuron dysfunction.

Cited by 55 publications

References 9 publications

Creatinine/cystatin C ratio as a surrogate marker of residual muscle mass in amyotrophic lateral sclerosis

Creatinine/cystatin C ratio as a surrogate marker of residual muscle mass in amyotrophic lateral sclerosis

Muscle Gene Expression Is a Marker of Amyotrophic Lateral Sclerosis Severity

Imagerie et spectroscopie par résonance magnétique nucléaire du muscle strié squelettique

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