{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T01:15:13Z","timestamp":1787015713628,"version":"build-2736575974"},"reference-count":41,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2022,4,8]],"date-time":"2022-04-08T00:00:00Z","timestamp":1649376000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Journal of Neuroimaging"],"published-print":{"date-parts":[[2022,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:label\/>\n                    <jats:p>Hirayama disease (HD) is a rare, benign, and nonprogressive motor neuron disease (MND) affecting the upper limbs. It usually presents with weakness and amyotrophy in a single upper extremity with an insidious onset between adolescence and the third decade of life. Since its description in 1959, HD has been known under several names and eponyms in Europe and in Asian countries probably due to its heterogeneous clinical features. Thus, the unclear nosological classification makes challenging the differential diagnosis between HD and other neuromuscular conditions, such as MNDs. However, apart from the nosological difficulties and the lack of evidence\u2010based guideline for diagnosis, the neuroimaging is the mainstay for the diagnosis of HD. Indeed, the specific findings on cervical flexion MRI usually lead to a prompt diagnosis. Here, we reviewed the nosological classifications of HD and its neuroimaging features. Also, we report a case of a 18\u2010year\u2010old boy who presented to our Clinic complaining of muscle weakness of the left distal upper limb without other neurological signs. The cervical MRI, in the neutral position, revealed a high T2 signal intensity in the C5\u2010C7 cervical myelomeres as well as the loss of cervical lordosis, whereas, during neck flexion, it showed the anterior displacement of the posterior dura ad the post\u2010gadolinium T1\u2010weighted imaging enhancement of the posterior epidural plexus. These findings are typical for HD allowing the diagnosis as well as the differential diagnosis from other neuromuscular diseases.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/jon.12995","type":"journal-article","created":{"date-parts":[[2022,4,8]],"date-time":"2022-04-08T11:44:39Z","timestamp":1649418279000},"page":"596-603","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Hirayama disease: Nosological classification and neuroimaging clues for diagnosis"],"prefix":"10.1111","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0035-5859","authenticated-orcid":false,"given":"Salvatore","family":"Iacono","sequence":"first","affiliation":[{"name":"Section of Neurology, Department of Biomedicine, Neuroscience, and Advanced Diagnostics (BiND) University of Palermo  Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincenzo","family":"Di Stefano","sequence":"additional","affiliation":[{"name":"Section of Neurology, Department of Biomedicine, Neuroscience, and Advanced Diagnostics (BiND) University of Palermo  Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Gagliardo","sequence":"additional","affiliation":[{"name":"Section of Neurology, Department of Biomedicine, Neuroscience, and Advanced Diagnostics (BiND) University of Palermo  Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Cannella","sequence":"additional","affiliation":[{"name":"Section of Radiology, Department of Biomedicine, Neuroscience and Advanced Diagnostics (BiND), University Hospital \u201cPaolo Giaccone\u201d University of Palermo  Palermo Italy"},{"name":"Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE) University of Palermo  Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Virz\u00ec","sequence":"additional","affiliation":[{"name":"U.F. Radiodiagnostica Casa di cura Regina Pacis  San Cataldo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonia","family":"Pagano","sequence":"additional","affiliation":[{"name":"Section of Neurology, Department of Biomedicine, Neuroscience, and Advanced Diagnostics (BiND) University of Palermo  Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonino","family":"Lupica","sequence":"additional","affiliation":[{"name":"Section of Neurology, Department of Biomedicine, Neuroscience, and Advanced Diagnostics (BiND) University of Palermo  Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcello","family":"Romano","sequence":"additional","affiliation":[{"name":"Neurology Unit Azienda Ospedaliera Ospedali Riuniti Villa Sofia Cervello  Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filippo","family":"Brighina","sequence":"additional","affiliation":[{"name":"Section of Neurology, Department of Biomedicine, Neuroscience, and Advanced Diagnostics (BiND) University of Palermo  Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,4,8]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.13.5.373"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.50.3.285"},{"key":"e_1_2_4_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nic.2011.07.009"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.39.283"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.5535\/arm.2015.39.1.122"},{"key":"e_1_2_4_7_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0404.2003.02142.x"},{"key":"e_1_2_4_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181aa5364"},{"key":"e_1_2_4_9_1","first-page":"471","article-title":"Benign monomelic amyotrophy in a 7\u2010year\u2010old girl with proximal upper limb 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