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This study identifies distinctive muscle MRI features to support diagnosis and guide molecular testing. Methods: Clinical, genetic and muscle MRI data were analyzed to separate DMs from dHMNs based on infiltration texture and distribution patterns. Results: dHMNs more often showed length-dependent involvement, distal-to-proximal fat replacement, and severe foot muscle involvement. Reticular fat infiltration was exclusive to dHMNs, while moth-eaten patterns predominated in DMs. Muscle islands were more common in dHMNs but also occurred in DMs. Conclusions: MRI feature analysis can differentiate DMs from dHMNs; reticular patterns appear early in dHMNs, and inclusion of foot muscles improves diagnostic yield.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/muscle-mri-contributes-to-the-differential-diagnosis-between-distal-myopathies-and-distal-hereditary-motor-neuropathies/457332/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/muscle-mri-contributes-to-the-differential-diagnosis-between-distal-myopathies-and-distal-hereditary-motor-neuropathies/457332.png","ImageObject",300,407,{"name":92,"@type":93},"Adam","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-31","2026-09-30",true,{"@type":102,"interactionType":103,"userInteractionCount":24},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"Why is differentiating distal myopathies from distal hereditary motor neuropathies challenging?","Question",{"text":112,"@type":113},"Clinical features, EMG findings and biopsy results sometimes overlap between DMs and dHMNs, which complicates determining whether the condition is myopathic or neurogenic.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"What MRI patterns were more common in dHMNs compared with DMs?",{"text":117,"@type":113},"dHMNs more often showed a length-dependent pattern of muscle involvement, a distal-to-proximal gradient of fat replacement, and severe, widespread involvement of foot muscles.",{"name":119,"@type":110,"acceptedAnswer":120},"Which MRI findings can help distinguish the two conditions and how is foot muscle imaging used?",{"text":121,"@type":113},"A reticular pattern of fat infiltration was exclusive to dHMNs, while DMs more often showed moth-eaten patterns. Muscle islands were common in advanced disease and in some DMs. The study recommends including foot muscles in MRI protocols because they are extensively involved in most dHMNs and correlate with greater fatty infiltration of lower leg muscles in DMs.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},457332,1791298471,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":24,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":139,"language":140,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":67,"update_tm":144,"read_time":41,"preview_page_count":4},1374404737137,"https://ap-avatar.wpscdn.com/davatar_155a257f0dc6eb9ab79c44ca47cae57d","European Journal of Neurology  \nORIGINAL ARTICLE  OPEN ACCESS   \nMuscle MRI Contributes to the Differential Diagnosis Between Distal Myopathies and Distal Hereditary Motor Neuropathies  \nMaría Payá1,2,3  | Sofía Portela1,2 | Rafael Sivera1,2,4,5,6 | Marina Frasquet1,2 | Fernando Más-Estellés7 | Pilar Martí4,5 | Inmaculada Azorín4,5 | Karolina Aragon-Gawinska4  | María Tárrega8 | Herminia Argente-Escrig1,2  |  \nJuan F. Vázquez-Costa1,2,4,5,9 | Roger Vilchez4,5 | Juan J. Vilchez1,2,4,5 | Teresa Sevilla1,2,4,5,9 | Nuria Muelas1,2,4,5,9   \n1Neuromuscular Diseases Unit, Neurology Department, Hospital Universitari I Politècnic La Fe, Valencia, Spain | 2Member of the European Reference Network for Rare Neuromuscular Diseases (ERN EURO-NMD) | 3Instituto de Investigación Sanitaria de Castilla-La Mancha (IDISCAM), Toledo, Spain | 4Neuromuscular and Ataxias Research Group, Instituto de Investigación Sanitaria La Fe, Valencia, Spain | 5Centro de Investigación Biomédicaen Red de Enfermedades Raras (CIBERER), Valencia, Spain | 6Department of Medicine, Universidad CEU-Cardenal Herrera, Alfara del Patriarca, Spain | 7Ascires, Neurorradiology Section, Área Clínica de Imagen Médica, Hospital Universitari I Politècnic La Fe, Valencia, Spain | 8Department of  \nClinical Neurophysiology, Hospital Universitari i Politècnic La Fe, Valencia, Spain | 9Department of Medicine, Universitat de València, Valencia, Spain Correspondence: Nuria Muelas ([nuria.muelas@uv.es](nuria.muelas@uv.es))  \nReceived: 3 July 2025 | Revised: 1 October 2025 | Accepted: 14 November 2025  \nKeywords: distal hereditary motor neuropathies | distal myopathies | muscle islands | muscle MRI | neuromyopathies  \nABSTRACT  \nBackground: Distinguishing between distal myopathies (DMs) and distal hereditary motor neuropathies (dHMNs) can be challenging because clinical, EMG and biopsy findings sometimes overlap. This study aims to identify distinctive muscle MRI features that can guide the diagnosis.  \nMethods: We collected clinical, genetic and muscle MRI data from patients with a confirmed diagnosis of DM and dHMN. We analyzed potential MRI characteristics to distinguish these conditions and to guide molecular diagnosis, such as the texture and pattern of infiltration.  \nResults: Seventy-eight (71.5%) patients diagnosed with DMs and thirty-one (28.4%) with dHMNs were included. A lengthdependent pattern of muscle involvement, a distal to proximal gradient of fat replacement along the length of the muscles and severe and widespread involvement of foot muscles were more common in patients with dHMNs. Muscle hypertrophy and asymmetry were more frequently observed in the DMs. A reticular pattern of fat infiltration was exclusive to patients with dHMNs, while the moth-eaten pattern predominated in DMs. Muscle islands were more commonly identified in dHMNs (54.8%) but were also observed in 32% of patients with DMs.  \nConclusions: Analysis of MRI features can help distinguish between DMs and dHMNs. A reticular pattern is an early feature of dHMNs while muscle islands are identified in advanced stages and in some forms of DMs, though not specific to neurogenic conditions. We recommend including foot muscles in the MRI protocol as they show extensive involvement in most dHMNs, while in DMs their involvement correlates with greater fatty infiltration of lower leg muscles.  \n\n| María Payá and Sofía Portela are contributed equally to this work. |\n| --- |\n| This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.\u003Cbr>© 2026 The Author(s). European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology. |\n\nEuropean Journal of Neurology, 2026; 33:e70456 1 of 12  \n[https://doi.org/10.1111/ene.70456](https://doi.org/10.1111/ene.70456)  \n1 | ","cbCaicJd4cAPAmCJ","https://ap.wps.com/l/cbCaicJd4cAPAmCJ","pdf",1235945,12,"English","# Abstract\n# Introduction","[{\"question\":\"Why is differentiating distal myopathies from distal hereditary motor neuropathies challenging?\",\"answer\":\"Clinical features, EMG findings and biopsy results sometimes overlap between DMs and dHMNs, which complicates determining whether the condition is myopathic or neurogenic.\"},{\"question\":\"What MRI patterns were more common in dHMNs compared with DMs?\",\"answer\":\"dHMNs more often showed a length-dependent pattern of muscle involvement, a distal-to-proximal gradient of fat replacement, and severe, widespread involvement of foot muscles.\"},{\"question\":\"Which MRI findings can help distinguish the two conditions and how is foot muscle imaging used?\",\"answer\":\"A reticular pattern of fat infiltration was exclusive to dHMNs, while DMs more often showed moth-eaten patterns. Muscle islands were common in advanced disease and in some DMs. The study recommends including foot muscles in MRI protocols because they are extensively involved in most dHMNs and correlate with greater fatty infiltration of lower leg muscles in DMs.\"}]","Muscle MRI Contributes to the Differential Diagnosis Between Distal Myopathies and Distal Hereditary Motor Neuropathies | PDF",1790748905]