Evaluation of inflammatory lesions over 2 years in facioscapulohumeral muscular dystrophy
| Autor: | Sofie Thurø Østergaard, John Vissing, Julia R. Dahlqvist, Freja Fornander, Else R. Danielsen, Carsten Thomsen, Josefine de Stricker Borch, Nanna S. Poulsen |
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| Rok vydání: | 2020 |
| Předmět: |
Adult
Male Muscle Strength Dynamometer Pathology medicine.medical_specialty Walk Test Inflammation Thigh 030218 nuclear medicine & medical imaging 03 medical and health sciences 0302 clinical medicine medicine Humans Facioscapulohumeral muscular dystrophy Longitudinal Studies Muscle Strength Muscular dystrophy Muscle Skeletal Leg medicine.diagnostic_test business.industry Magnetic resonance imaging Muscle inflammation Middle Aged medicine.disease Magnetic Resonance Imaging Muscular Dystrophy Facioscapulohumeral Hyperintensity medicine.anatomical_structure Adipose Tissue Disease Progression Female Neurology (clinical) medicine.symptom business 030217 neurology & neurosurgery |
| Zdroj: | Neurology. 95:e1211-e1221 |
| ISSN: | 1526-632X 0028-3878 |
| Popis: | ObjectiveWe followed up patients with facioscapulohumeral muscular dystrophy (FSHD) with sequential examinations over 2 years to investigate whether inflammatory lesions always precede fat replacement, if inflammation can be resolved without muscle degeneration, and if inflammatory lesions in muscle are always followed by fat replacement.MethodsIn this longitudinal study of 10 sequential MRI assessments over 2.5 years, we included 10 patients with FSHD. We used MRI with short TI inversion recovery to identify regions of interest (ROIs) with hyperintensities indicating muscle inflammation. Muscle T2 relaxation time mapping was used as a quantitative marker of muscle inflammation. Dixon sequences quantified muscle fat replacement. Ten healthy controls were examined with a magnetic resonance scan once for determination of normal values of T2 relaxation time.ResultsWe identified 68 ROIs with T2 elevation in the patients with FSHD. New ROIs with T2 elevation arising during the study had muscle fat content of 6.4% to 33.0% (n = 8) and 47.0% to 78.0% lesions that resolved (n = 6). ROIs with T2 elevation had a higher increase in muscle fat content from visits 1 to 10 (7.9 ± 7.9%) compared to ROIs with normal muscle T2 relaxation times (1.7 ± 2.6%; p < 0.0001). Severe T2 elevations were always followed by an accelerated replacement of muscle by fat.ConclusionsOur results suggest that muscle inflammation starts in mildly affected muscles in FSHD, is related to a faster muscle degradation, and continues until the muscles are completely fat replaced.ClinicalTrials.gov identifierNCT02159612. |
| Databáze: | OpenAIRE |
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