Journal article
Quantitative Foot Muscle Magnetic Resonance Imaging Reliably Measures Disease Progression in Children and Adolescents with Charcot-Marie-Tooth Disease Type 1A
Annals of neurology, Vol.96(1), pp.170-174
07/2024
DOI: 10.1002/ana.26934
PMID: 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.
Details
- Title: Subtitle
- Quantitative Foot Muscle Magnetic Resonance Imaging Reliably Measures Disease Progression in Children and Adolescents with Charcot-Marie-Tooth Disease Type 1A
- Creators
- Carolynne M Doherty - University College LondonPaige Howard - University of IowaLuke F O'Donnell - National Hospital for Neurology and NeurosurgeryRiccardo Zuccarino - University of IowaStephen Wastling - National Hospital for Neurology and NeurosurgeryEvelin Milev - Great Ormond Street HospitalTina Banks - Great Ormond Street HospitalSachit Shah - National Hospital for Neurology and NeurosurgeryNick Zafeiropoulos - National Hospital for Neurology and NeurosurgeryKatherine J Stephens - Roy and Lucille Carver College of Medicine, University of Iowa, Iowa City, IA, USAAnna Sarkozy - Great Ormond Street HospitalTiffany Grider - University of IowaShawna M E Feely - University of Washington School of MedicineAdnan Manzur - University College LondonRosemary R Shy - University of IowaMariola Skorupinska - National Hospital for Neurology and NeurosurgeryMenelaos Pipis - National Hospital for Neurology and NeurosurgeryEmma Nicolaisen - University of IowaAmy McDowell - National Hospital for Neurology and NeurosurgeryNuran Dilek - University of RochesterAlexander M Rossor - National Hospital for Neurology and NeurosurgeryMatilde Laura - National Hospital for Neurology and NeurosurgeryChristopher Clark - University College LondonFrancesco Muntoni - Great Ormond Street HospitalDaniel Thedens - University of IowaJohn Thornton - National Hospital for Neurology and NeurosurgeryJasper M Morrow - National Hospital for Neurology and NeurosurgeryMichael E Shy - University of IowaMary M Reilly - National Hospital for Neurology and Neurosurgery
- Resource Type
- Journal article
- Publication Details
- Annals of neurology, Vol.96(1), pp.170-174
- DOI
- 10.1002/ana.26934
- PMID
- 38613459
- NLM abbreviation
- Ann Neurol
- ISSN
- 0364-5134
- eISSN
- 1531-8249
- Grant note
- MDA510281 / Muscular Dystrophy Association
- Language
- English
- Electronic publication date
- 04/13/2024
- Date published
- 07/2024
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Neurology; Radiology; Electrical and Computer Engineering; Molecular Physiology and Biophysics; Iowa Neuroscience Institute
- Record Identifier
- 9984616955202771
Metrics
6 Record Views