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Quantitative Foot Muscle Magnetic Resonance Imaging Reliably Measures Disease Progression in Children and Adolescents with Charcot-Marie-Tooth Disease Type 1A.
Doherty, Carolynne M; Howard, Paige; O'Donnell, Luke F; Zuccarino, Riccardo; Wastling, Stephen; Milev, Evelin; Banks, Tina; Shah, Sachit; Zafeiropoulos, Nick; Stephens, Katherine J; Sarkozy, Anna; Grider, Tiffany; Feely, Shawna M E; Manzur, Adnan; Shy, Rosemary R; Skorupinska, Mariola; Pipis, Menelaos; Nicolaisen, Emma; McDowell, Amy; Dilek, Nuran; Rossor, Alexander M; Laura, Matilde; Clark, Christopher; Muntoni, Francesco; Thedens, Daniel; Thornton, John; Morrow, Jasper M; Shy, Michael E; Reilly, Mary M.
Afiliação
  • Doherty CM; Center for Neuromuscular Diseases, Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, UK.
  • Howard P; Roy and Lucille Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
  • O'Donnell LF; Center for Neuromuscular Diseases, Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, UK.
  • Zuccarino R; Roy and Lucille Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
  • Wastling S; Fondazione Serena Onlus, Centro Clinico NeMO Trento, Italy.
  • Milev E; Lysholm Department of Radiology, National Hospital for Neurology and Neurosurgery, London, UK.
  • Banks T; Dubowitz Neuromuscular Center, Great Ormond Street Hospital, London, UK.
  • Shah S; Department of Radiology, Great Ormond Street Hospital, London, UK.
  • Zafeiropoulos N; Lysholm Department of Radiology, National Hospital for Neurology and Neurosurgery, London, UK.
  • Stephens KJ; Lysholm Department of Radiology, National Hospital for Neurology and Neurosurgery, London, UK.
  • Sarkozy A; Roy and Lucille Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
  • Grider T; Dubowitz Neuromuscular Center, Great Ormond Street Hospital, London, UK.
  • Feely SME; Roy and Lucille Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
  • Manzur A; Division of Pediatric Neurology, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, WA, USA.
  • Shy RR; Dubowitz Neuromuscular Center, Great Ormond Street Hospital, London, UK.
  • Skorupinska M; Roy and Lucille Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
  • Pipis M; Center for Neuromuscular Diseases, Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, UK.
  • Nicolaisen E; Center for Neuromuscular Diseases, Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, UK.
  • McDowell A; Roy and Lucille Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
  • Dilek N; Center for Neuromuscular Diseases, Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, UK.
  • Rossor AM; Lysholm Department of Radiology, National Hospital for Neurology and Neurosurgery, London, UK.
  • Laura M; University of Rochester School of Medicine and Dentistry, New York, NY, USA.
  • Clark C; Center for Neuromuscular Diseases, Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, UK.
  • Muntoni F; Center for Neuromuscular Diseases, Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, UK.
  • Thedens D; Department of Radiology, Great Ormond Street Hospital, London, UK.
  • Thornton J; Dubowitz Neuromuscular Center, Great Ormond Street Hospital, London, UK.
  • Morrow JM; Roy and Lucille Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
  • Shy ME; Lysholm Department of Radiology, National Hospital for Neurology and Neurosurgery, London, UK.
  • Reilly MM; Center for Neuromuscular Diseases, Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, UK.
Ann Neurol ; 96(1): 170-174, 2024 Jul.
Article em En | MEDLINE | ID: mdl-38613459
ABSTRACT
Quantitative muscle fat fraction (FF) responsiveness is lower in younger Charcot-Marie-Tooth disease type 1A (CMT1A) patients with lower baseline calf-level FF. We investigated the practicality, validity, and responsiveness of foot-level FF in this cohort involving 22 CMT1A patients and 14 controls. The mean baseline foot-level FF was 25.9 ± 20.3% in CMT1A patients, and the 365-day FF (n = 15) increased by 2.0 ± 2.4% (p < 0.001 vs controls). Intrinsic foot-level FF demonstrated large responsiveness (12-month standardized response mean (SRM) of 0.86) and correlated with the CMT examination score (ρ = 0.58, P = 0.01). Intrinsic foot-level FF has the potential to be used as a biomarker in future clinical trials involving younger CMT1A patients. ANN NEUROL 2024;96170-174.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Imageamento por Ressonância Magnética / Doença de Charcot-Marie-Tooth / Músculo Esquelético / Progressão da Doença / Pé Limite: Adolescent / Adult / Child / Female / Humans / Male Idioma: En Revista: Ann Neurol Ano de publicação: 2024 Tipo de documento: Article
Texto completo
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PubMed Links
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Imageamento por Ressonância Magnética / Doença de Charcot-Marie-Tooth / Músculo Esquelético / Progressão da Doença / Pé Limite: Adolescent / Adult / Child / Female / Humans / Male Idioma: En Revista: Ann Neurol Ano de publicação: 2024 Tipo de documento: Article