{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T11:22:14Z","timestamp":1784546534287,"version":"3.55.0"},"reference-count":8,"publisher":"Georg Thieme Verlag KG","issue":"04","license":[{"start":{"date-parts":[[2022,8,10]],"date-time":"2022-08-10T00:00:00Z","timestamp":1660089600000},"content-version":"vor","delay-in-days":9,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Rev Bras Ortop (Sao Paulo)"],"published-print":{"date-parts":[[2022,8]]},"abstract":"<jats:title>Resumo<\/jats:title><jats:p>Paciente de 26 anos, previamente h\u00edgido, que, aos 18 anos, iniciou perda progressiva de for\u00e7a distal, tremor de repouso, e atrofia muscular no membro superior esquerdo. Ao exame, apresentou atrofia moderada, distal, for\u00e7a muscular de grau 4, e minipolimioclonus. A eletroneuromiografia (ENMG) revelou comprometimento pr\u00e9-ganglionar cr\u00f4nico de C7\/C8\/T1 bilateral pior \u00e0 esquerda, com sinais de desnerva\u00e7\u00e3o ativa em C8\/T1. A resson\u00e2ncia magn\u00e9tica (RM) de coluna cervical mostrou altera\u00e7\u00f5es degenerativas espondilodiscais com protrus\u00f5es centrais em C4-C5, C6-C7, e central direita em C5-C6, que tocavam o saco dural. O di\u00e2metro anteroposterior da medula na posi\u00e7\u00e3o neutra, no plano de C5-C6, era de 5,1\u2009mm. Houve redu\u00e7\u00e3o do calibre da medula para 4,0\u2009mm ap\u00f3s a manobra din\u00e2mica de flex\u00e3o for\u00e7ada da coluna, e aumento de sinal nos cornos anteriores. Os achados cl\u00ednicos e os dos exames complementares eram compat\u00edveis com doen\u00e7a de Hirayama (DH), uma doen\u00e7a benigna rara dos neur\u00f4nios motores, que afeta os segmentos espinhais cervicais e \u00e9 mais prevalente em homens e de in\u00edcio pr\u00f3ximo aos 20 anos. \u00c9 t\u00edpica a fraqueza unilateral e lentamente progressiva, por\u00e9m autolimitada. Perturba\u00e7\u00f5es sensoriais, sinais auton\u00f4micos e do neur\u00f4nio motor superior s\u00e3o raras.<\/jats:p><jats:p>O manejo geralmente \u00e9 conservador, com uso de colar cervical macio. Apesar de rara, a DH deve ser considerada em pacientes jovens que apresentam atrofias assim\u00e9tricas focais de membros superiores. O diagn\u00f3stico precoce de DH depende do grau de suspei\u00e7\u00e3o, e da coopera\u00e7\u00e3o e comunica\u00e7\u00e3o entre as diversas especialidades envolvidas na investiga\u00e7\u00e3o.<\/jats:p>","DOI":"10.1055\/s-0042-1742339","type":"journal-article","created":{"date-parts":[[2022,8,10]],"date-time":"2022-08-10T22:38:02Z","timestamp":1660171082000},"page":"697-701","source":"Crossref","is-referenced-by-count":1,"title":["Doen\u00e7a de Hirayama: Relato de caso*"],"prefix":"10.1055","volume":"57","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8606-6809","authenticated-orcid":false,"given":"Victor Alves","family":"Rodrigues","sequence":"additional","affiliation":[{"name":"M\u00e9dico Neurologista Pedi\u00e1trico do Hospital Universit\u00e1rio de Bras\u00edlia, Bras\u00edlia, DF, Brasil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matheus Rocha Pereira","family":"Klettenberg","sequence":"additional","affiliation":[{"name":"M\u00e9dico Neurologista Pedi\u00e1trico do Hospital Universit\u00e1rio de Bras\u00edlia, Bras\u00edlia, DF, Brasil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luciano","family":"Farage","sequence":"additional","affiliation":[{"name":"M\u00e9dico Radiologista do Incor, Bras\u00edlia, DF, Brasil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisiane","family":"Seguti","sequence":"additional","affiliation":[{"name":"M\u00e9dico Neurologista Pedi\u00e1trico do Hospital Universit\u00e1rio de Bras\u00edlia, Bras\u00edlia, DF, Brasil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2022,8,10]]},"reference":[{"issue":"04","key":"ref1","doi-asserted-by":"crossref","first-page":"283","DOI":"10.2169\/internalmedicine.39.283","article-title":"Juvenile muscular atrophy of distal upper extremity (Hirayama disease)","volume":"39","author":"K Hirayama","year":"2000","journal-title":"Intern Med"},{"issue":"01","key":"ref2","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1080\/14660820500396877","article-title":"Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan","volume":"7","author":"K Tashiro","year":"2006","journal-title":"Amyotroph Lateral Scler"},{"issue":"04","key":"ref3","doi-asserted-by":"crossref","first-page":"364","DOI":"10.21037\/qims.2016.07.08","article-title":"Hirayama's disease: an Italian single center experience and review of the literature","volume":"6","author":"V Vitale","year":"2016","journal-title":"Quant Imaging Med Surg"},{"issue":"13","key":"ref4","doi-asserted-by":"crossref","first-page":"58","DOI":"10.17161\/kjm.v13i1.13530","article-title":"Significance of Dynamic Imaging in Diagnosis of Hirayama Disease: A Rare Case Report and Literature Review","volume":"13","author":"G R Kandukuri","year":"2020","journal-title":"Kans J Med"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"478516","DOI":"10.1155\/2013\/478516","article-title":"Nosology of juvenile muscular atrophy of distal upper extremity: from monomelic amyotrophy to Hirayama disease\u2013Indian perspective","volume":"2013","author":"K M Hassan","year":"2013","journal-title":"BioMed Res Int"},{"issue":"04","key":"ref6","doi-asserted-by":"crossref","first-page":"245","DOI":"10.4103\/0971-3026.73528","article-title":"MRI findings in Hirayama disease","volume":"20","author":"M Raval","year":"2010","journal-title":"Indian J Radiol Imaging"},{"issue":"04","key":"ref7","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1016\/j.nic.2011.07.009","article-title":"Hirayama disease","volume":"21","author":"Y L Huang","year":"2011","journal-title":"Neuroimaging Clin N Am"},{"key":"ref8","first-page":"173","article-title":"[Cervical collar therapy for juvenile muscular atrophy of distal upper extremity (Hirayama disease): results from 38 cases]","volume":"41","author":"Y Tokumaru","year":"2001","journal-title":"Rinsho Shinkeigaku"}],"container-title":["Revista Brasileira de Ortopedia"],"original-title":[],"language":"pt","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/s-0042-1742339.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,8,10]],"date-time":"2022-08-10T22:45:54Z","timestamp":1660171554000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/s-0042-1742339"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8]]},"references-count":8,"journal-issue":{"issue":"04","published-online":{"date-parts":[[2022,8,10]]},"published-print":{"date-parts":[[2022,8]]}},"URL":"https:\/\/doi.org\/10.1055\/s-0042-1742339","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0102-3616","1982-4378"],"issn-type":[{"value":"0102-3616","type":"print"},{"value":"1982-4378","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,8]]}}}