{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,16]],"date-time":"2025-12-16T00:27:36Z","timestamp":1765844856380,"version":"3.48.0"},"reference-count":14,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2025,11,15]],"date-time":"2025-11-15T00:00:00Z","timestamp":1763164800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2025,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Immune\u2010mediated necrotizing myopathy (IMNM) is a frequent subtype of idiopathic inflammatory myopathies. Distinguishing juvenile\u2010onset IMNM from muscular dystrophy is often difficult, particularly when the disorder takes a chronic course. We describe a 45\u2010year\u2010old Japanese woman with progressive generalized weakness. Her early motor milestones were normal until age 4, but by 5\u2009years she developed Gowers' sign. At that time, she was noted to have proximal weakness, scapular winging, gait impairment, and markedly elevated serum creatine kinase (6069\u2009IU\/L). A biopsy of the left biceps brachii suggested muscular dystrophy. Her disease advanced, and she required a wheelchair during elementary school. At age 41 she was referred to the neurology department at Tottori University Hospital, and at 45\u2009years she was re\u2010evaluated. Examination revealed symmetric proximal weakness, atrophy, severe spinal deformity, and bilateral pes cavus. Electromyography demonstrated prominent myogenic changes, and muscle MRI disclosed profound atrophy with fatty replacement of proximal muscles. A repeat biopsy from the left tibialis anterior showed severe myofiber atrophy but no necrosis, regeneration, or inflammatory infiltration. Immunohistochemistry for dystrophy\u2010associated proteins, MHC class I, and p62 was unremarkable. In contrast, retrospective review of the original biopsy revealed diffuse MHC class I expression and granular sarcoplasmic p62 staining, supporting a diagnosis of IMNM. Although ELISA results were negative, RNA immunoprecipitation identified anti\u2010signal recognition particle antibodies. This patient illustrates an uncommon presentation of juvenile\u2010onset IMNM that remained untreated for decades. The absence of characteristic histological features on biopsy performed 40\u2009years after disease onset emphasizes the diagnostic challenges in long\u2010standing cases. Careful reassessment of earlier tissue samples, in combination with extended antibody testing, may be essential for establishing the correct diagnosis in similar situations.<\/jats:p>","DOI":"10.1111\/neup.70034","type":"journal-article","created":{"date-parts":[[2025,11,15]],"date-time":"2025-11-15T10:36:37Z","timestamp":1763202997000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Anti\u2010Signal Recognition Particle Antibody\u2010Positive Immune\u2010Mediated Necrotizing Myopathy Diagnosed by Re\u2010Evaluation 40\u2009Years After Symptom Onset"],"prefix":"10.1111","volume":"45","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3053-4033","authenticated-orcid":false,"given":"Kentaro","family":"Yoshida","sequence":"first","affiliation":[{"name":"Division of Neurology, Department of Brain and Neuroscience Tottori University  Yonago Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4919-2325","authenticated-orcid":false,"given":"Tadashi","family":"Adachi","sequence":"additional","affiliation":[{"name":"Division of Neuropathology, Department of Brain and Neuroscience Tottori University  Yonago Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shigeaki","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Neurology Tokyo Metropolitan Neurological Hospital  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9452-112X","authenticated-orcid":false,"given":"Ichizo","family":"Nishino","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Research National Institute of Neuroscience, National Center of Neurology and Psychiatry  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ritsuko","family":"Hanajima","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Brain and Neuroscience Tottori University  Yonago Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,11,15]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2018.2598"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2016-313166"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000004923"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2018-213518"},{"key":"e_1_2_11_6_1","first-page":"3","article-title":"Juvenile Idiopathic Inflammatory Myopathy: A Muscle Pathology Perspective","volume":"12","author":"Inoue M.","year":"2021","journal-title":"Journal of Clinical Pediatric Rheumatology"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kew386"},{"key":"e_1_2_11_8_1","article-title":"Clinical Course, Myopathology and Challenge of Therapeutic Intervention in Pediatric Patients With Autoimmune\u2010Mediated Necrotizing Myopathy","volume":"8","author":"Marina A. D.","year":"2021","journal-title":"Children (Basel)"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2023.1123380"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-015-0277-y"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/ken325"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2011.04.009"},{"key":"e_1_2_11_13_1","first-page":"927","article-title":"Autoantibody to Signal Recognition Particle in African American Girls With Juvenile Polymyositis","volume":"35","author":"Rouster\u2010Stevens K. A.","year":"2008","journal-title":"Journal of Rheumatology"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.25575"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.3390\/diagnostics13182929"}],"container-title":["Neuropathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/neup.70034","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,16]],"date-time":"2025-12-16T00:26:19Z","timestamp":1765844779000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/neup.70034"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,15]]},"references-count":14,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2025,12]]}},"alternative-id":["10.1111\/neup.70034"],"URL":"https:\/\/doi.org\/10.1111\/neup.70034","archive":["Portico"],"relation":{},"ISSN":["0919-6544","1440-1789"],"issn-type":[{"type":"print","value":"0919-6544"},{"type":"electronic","value":"1440-1789"}],"subject":[],"published":{"date-parts":[[2025,11,15]]},"assertion":[{"value":"2025-07-22","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-11-10","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-11-15","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70034"}}