Skeletal muscle MRI differentiates SBMA and ALS and correlates with disease severity
| Autor: | Pietro Fratta, Andrea Malaspina, Robin Howard, Karin Trimmel, Luca Zampedri, Ahmed Emira, Nikhil Sharma, John S. Thornton, Jasper M. Morrow, Linda Greensmith, Tarek A. Yousry, Katie Sidle, Stephen J. Wastling, Uros Klickovic, Michael G. Hanna, Christopher D. J. Sinclair, Sachit Shah |
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| Jazyk: | angličtina |
| Rok vydání: | 2019 |
| Předmět: |
0301 basic medicine
Male Pathology medicine.medical_specialty Thigh Severity of Illness Index Article 030218 nuclear medicine & medical imaging Diagnosis Differential Muscular Atrophy Spinal 03 medical and health sciences 0302 clinical medicine Atrophy Severity of illness medicine Humans Prospective Studies Amyotrophic lateral sclerosis Muscle Skeletal medicine.diagnostic_test business.industry Amyotrophic Lateral Sclerosis Skeletal muscle Magnetic resonance imaging Motor neuron Middle Aged medicine.disease Magnetic Resonance Imaging Hyperintensity 030104 developmental biology medicine.anatomical_structure Cross-Sectional Studies Case-Control Studies Biomarker (medicine) Female Neurology (clinical) Differential diagnosis business 030217 neurology & neurosurgery |
| Zdroj: | Neurology |
| ISSN: | 1526-632X 0028-3878 |
| Popis: | ObjectiveTo investigate the use of muscle MRI for the differential diagnosis and as a disease progression biomarker for 2 major forms of motor neuron disorders: spinal bulbar muscular atrophy (SBMA) and amyotrophic lateral sclerosis (ALS).MethodsWe applied quantitative 3-point Dixon and semiquantitative T1-weighted and short tau inversion recovery (STIR) imaging to bulbar and lower limb muscles and performed clinical and functional assessments in ALS (n = 21) and SBMA (n = 21), alongside healthy controls (n = 16). Acquired images were analyzed for the presence of fat infiltration or edema as well as specific patterns of muscle involvement. Quantitative MRI measurements were correlated with clinical measures of disease severity in ALS and SBMA.ResultsQuantitative imaging revealed significant fat infiltration in bulbar (p < 0.001) and limb muscles in SBMA compared to controls (thigh: p < 0.001; calf: p = 0.001), identifying a characteristic pattern of muscle involvement. In ALS, semiquantitative STIR imaging detected marked hyperintensities in lower limb muscles, distinguishing ALS from SBMA and controls. Finally, MRI measurements correlated significantly with clinical scales of disease severity in both ALS and SBMA.ConclusionsOur findings show that muscle MRI differentiates between SBMA and ALS and correlates with disease severity, supporting its use as a diagnostic tool and biomarker for disease progression. This highlights the clinical utility of muscle MRI in motor neuron disorders and contributes to establish objective outcome measures, which is crucial for the development of new drugs. |
| Databáze: | OpenAIRE |
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