Search Results - "De Wel, Bram"

Nusinersen treatment significantly improves hand grip strength, hand motor function and MRC sum scores in adult patients with spinal muscular atrophy types 3 and 4 by De Wel, Bram, Goosens, Veerle, Sobota, Atka, Van Camp, Elke, Geukens, Ellen, Van Kerschaver, Griet, Jagut, Marlène, Claes, Kathleen, Claeys, Kristl G.

“…Background Nusinersen recently became available as the first treatment for Spinal Muscular Atrophy (SMA) and data on its effectiveness and safety in adult SMA…”
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Automated MRI quantification of volumetric per-muscle fat fractions in the proximal leg of patients with muscular dystrophies by Huysmans, Lotte, De Wel, Bram, Claeys, Kristl G, Maes, Frederik

“…Muscular dystrophies (MD) are a class of rare genetic diseases resulting in progressive muscle weakness affecting specific muscle groups, depending on the type…”
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Lessons for future clinical trials in adults with Becker muscular dystrophy: Disease progression detected by muscle magnetic resonance imaging, clinical and patient‐reported outcome measures by De Wel, Bram, Iterbeke, Louise, Huysmans, Lotte, Peeters, Ronald, Goosens, Veerle, Dubuisson, Nicolas, Bergh, Peter, Van Parijs, Vinciane, Remiche, Gauthier, De Waele, Liesbeth, Maes, Frederik, Dupont, Patrick, Claeys, Kristl G.

“…Background and purpose Because Becker muscular dystrophy (BMD) is a heterogeneous disease and only few studies have evaluated adult patients, it is currently…”
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Biochemical and clinical biomarkers in adult SMA 3–4 patients treated with nusinersen for 22 months by De Wel, Bram, De Schaepdryver, Maxim, Poesen, Koen, Claeys, Kristl G.

“…Objective To investigate biomarkers of disease progression in cerebrospinal fluid (CSF) and serum in adult patients with spinal muscular atrophy (SMA)…”
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Test–retest reliability and follow‐up of muscle magnetic resonance elastography in adults with and without muscle diseases by De Wel, Bram, Huysmans, Lotte, Peeters, Ronald, Ghysels, Stefan, Byloos, Kris, Putzeys, Guido, Maes, Frederik, Dupont, Patrick, Claeys, Kristl G.

“…Background We investigated the potential of magnetic resonance elastography (MRE) stiffness measurements in skeletal muscles as an outcome measure, by…”
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Histopathological correlations and fat replacement imaging patterns in recessive limb‐girdle muscular dystrophy type 12 by De Wel, Bram, Huysmans, Lotte, Depuydt, Christophe E., Goosens, Veerle, Peeters, Ronald, Santos, Filipa P., Thal, Dietmar R., Dupont, Patrick, Maes, Frederik, Claeys, Kristl G.

“…Background Despite the widespread use of proton density fat fraction (PDFF) measurements with magnetic resonance imaging (MRI) to track disease progression in…”
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Author Response: Prospective Natural History Study in 24 Adult Patients With LGMDR12 Over 2 Years of Follow-up: Quantitative MRI and Clinical Outcome Measures by De Wel, Bram, Claeys, Kristl G.

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Neuromuscular hyperexcitability syndromes by De Wel, Bram, Claeys, Kristl G.

“…To provide an update on recent developments regarding acquired, antibody-mediated, neuromuscular hyperexcitability syndromes, including Isaac's and Morvan's…”
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Malignant hyperthermia: still an issue for neuromuscular diseases? by De Wel, Bram, Claeys, Kristl G

“…We will give an overview of neuromuscular disorders that can be linked with malignant hyperthermia or malignant hyperthermia-like reactions, and suggest an…”
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Prospective Natural History Study in 24 Adult Patients With LGMDR12 Over 2 Years of Follow-up: Quantitative MRI and Clinical Outcome Measures by De Wel, Bram, Huysmans, Lotte, Peeters, Ronald, Goosens, Veerle, Ghysels, Stefan, Byloos, Kris, Putzeys, Guido, D'Hondt, Ann, De Bleecker, Jan L., Dupont, Patrick, Maes, Frederik, Claeys, Kristl G.

“…Limb-girdle muscular dystrophy autosomal recessive type 12 (LGMDR12) is a rare hereditary muscular dystrophy for which outcome measures are currently lacking…”
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Prospective Natural History Study in 24 Adult Patients With LGMDR12 Over 2 Years’ Follow-up: Quantitative MRI and Clinical Outcome Measures by De Wel, Bram, Huysmans, Lotte, Peeters, Ronald, Goosens, Veerle, Ghysels, Sfefan, Byloos, Kris, Putzeys, Guido, D'Hondt, Ann, De Bleecker, Jan L, Dupont, Patrick, Maes, Frederik, Claeys, Kristl G

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Respiratory decline in adult patients with Becker muscular dystrophy: A longitudinal study by De Wel, Bram, Willaert, Sofie, Nadaj-Pakleza, Aleksandra, Aubé-Nathier, Anne-Catherine, Testelmans, Dries, Buyse, Bertien, Claeys, Kristl G.

“…•FVC% predicted significantly declines with 1% per year in adult BMD patients.•FEV1 and PEF also deteriorate significantly each year.•Loss of ambulation…”
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