{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T18:08:18Z","timestamp":1777486098578,"version":"3.51.4"},"reference-count":9,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2020,8,21]],"date-time":"2020-08-21T00:00:00Z","timestamp":1597968000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2020,8,21]],"date-time":"2020-08-21T00:00:00Z","timestamp":1597968000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Med Case Reports"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n<jats:title>Background<\/jats:title>\n<jats:p>Magnetic resonance imaging (MRI) features are typical findings in Hirayama disease (HD) and are useful diagnostic entities but may not be present in all patients.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Case presentation<\/jats:title>\n<jats:p>We present the case of a 22-year-old Nepalese man who presented with insidious onset of weakness of his right upper limb of more than 5 years duration. His weakness was progressive for the first 3\u2009years, and then remained static. On examination, weakness of the interossei, thenar, hypothenar, flexor, and extensor muscles were present in his right upper limb, power was normal in his left upper and bilateral lower limbs. Minipolymyoclonus was present in both upper limbs, less prominent on the left side. Electrophysiological findings showed motor axonal neuropathy in his right upper limb, neurogenic discharges and fibrillations, and fasciculations in both upper limbs. Contrast magnetic resonance imaging (MRI) of his cervical spine in flexion was normal. Our patient was diagnosed with HD based on clinical and electrophysiological findings. Our patient was advised to use a cervical collar and regular physiotherapy and was found to have subjective benefit.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Conclusion<\/jats:title>\n<jats:p>A normal cervical MRI does not rule out HD and the diagnosis can also be made based on clinical and electrophysiological studies. Progressive distal upper limb weakness or tremor in young patients should be evaluated for HD, because early diagnosis and intervention might halt the progression.<\/jats:p>\n<\/jats:sec>","DOI":"10.1186\/s13256-020-02453-2","type":"journal-article","created":{"date-parts":[[2020,8,20]],"date-time":"2020-08-20T23:03:59Z","timestamp":1597964639000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["The diagnostic quandary of magnetic resonance imaging-negative Hirayama disease: a case report"],"prefix":"10.1186","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7680-7036","authenticated-orcid":false,"given":"Rajeev","family":"Ojha","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sumit","family":"Shahi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gaurav","family":"Nepal","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arjana","family":"Shakya","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bikram Prasad","family":"Gajurel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ragesh","family":"Karn","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Reema","family":"Rajbhandari","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Niraj","family":"Gautam","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2020,8,21]]},"reference":[{"issue":"1","key":"2453_CR1","first-page":"17","volume":"60","author":"K Hirayama","year":"2008","unstructured":"Hirayama K. Juvenile muscular atrophy of unilateral upper extremity (Hirayama disease) - Half-century progress and establishment since its discovery. Brain Nerve. 2008;60(1):17\u201329.","journal-title":"Brain Nerve"},{"issue":"1","key":"2453_CR2","doi-asserted-by":"publisher","first-page":"120","DOI":"10.1001\/archneur.62.1.120","volume":"62","author":"UK Misra","year":"2005","unstructured":"Misra UK, Kalita J, Mishra VN, Kesari A, Mittal B. A clinical, magnetic resonance imaging, and survival motor neuron gene deletion study of Hirayama disease. Arch Neurol. 2005;62(1):120\u20133.","journal-title":"Arch Neurol"},{"key":"2453_CR3","doi-asserted-by":"crossref","unstructured":"Li T, Alberman E, Swash M. Clinical features and associations of 560 cases of motor neuron disease. J Neurol Neurosurg Psychiatry. 1990;53(12):1043-45.","DOI":"10.1136\/jnnp.53.12.1043"},{"issue":"3","key":"2453_CR4","doi-asserted-by":"publisher","first-page":"170","DOI":"10.14245\/kjs.2016.13.3.170","volume":"13","author":"KH Lee","year":"2016","unstructured":"Lee KH, Choi DS, Lee YS, Kang DH. Clinical experiences of uncommon motor neuron disease: Hirayama disease. Korean J Spine. 2016;13(3):170.","journal-title":"Korean J Spine"},{"issue":"1","key":"2453_CR5","doi-asserted-by":"publisher","first-page":"79","DOI":"10.2169\/internalmedicine.43.79","volume":"43","author":"S Chiba","year":"2004","unstructured":"Chiba S, Yonekura K, Nonaka M, Imai T, Matumoto H, Wada T. Advanced Hirayama disease with successful improvement of activities of daily living by operative reconstruction. Intern Med. 2004;43(1):79\u201381.","journal-title":"Intern Med"},{"issue":"4","key":"2453_CR6","doi-asserted-by":"publisher","first-page":"245","DOI":"10.4103\/0971-3026.73528","volume":"20","author":"M Raval","year":"2010","unstructured":"Raval M, Kumari R, Dung Dung A, Guglani B, Gupta N, Gupta R. MRI findings in Hirayama disease. Indian J Radiol Imaging. 2010;20(4):245\u20139.","journal-title":"Indian J Radiol Imaging"},{"issue":"5","key":"2453_CR7","doi-asserted-by":"publisher","first-page":"974","DOI":"10.3174\/ajnr.A5577","volume":"39","author":"DK Boruah","year":"2018","unstructured":"Boruah DK, Prakash A, Gogoi BB, Yadav RR, Dhingani DD, Sarma B. The importance of flexion MRI in Hirayama disease with special reference to laminodural space measurements. Am J Neuroradiol. 2018;39(5):974\u201380.","journal-title":"Am J Neuroradiol"},{"key":"2453_CR8","doi-asserted-by":"publisher","first-page":"1385","DOI":"10.2147\/NDT.S138315","volume":"13","author":"H Aay","year":"2017","unstructured":"Aay H. Hirayama disease (monomelic amyotrophy) clinically confused for carpal tunnel syndrome. Neuropsychiatr Dis Treat. 2017;13:1385\u20138.","journal-title":"Neuropsychiatr Dis Treat"},{"issue":"1","key":"2453_CR9","doi-asserted-by":"publisher","first-page":"8","DOI":"10.1016\/j.cegh.2016.07.003","volume":"5","author":"AK Verma","year":"2017","unstructured":"Verma AK, Raj J, Sharma V, Singh TB, Srivastava S, Srivastava R. Epidemiology and associated risk factors of Parkinson\u2019s disease among the north Indian population. Clin Epidemiol Glob Heal. 2017;5(1):8\u201313.","journal-title":"Clin Epidemiol Glob Heal"}],"container-title":["Journal of Medical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13256-020-02453-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13256-020-02453-2\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13256-020-02453-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,8,20]],"date-time":"2021-08-20T23:22:48Z","timestamp":1629501768000},"score":1,"resource":{"primary":{"URL":"https:\/\/jmedicalcasereports.biomedcentral.com\/articles\/10.1186\/s13256-020-02453-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,8,21]]},"references-count":9,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2020,12]]}},"alternative-id":["2453"],"URL":"https:\/\/doi.org\/10.1186\/s13256-020-02453-2","relation":{},"ISSN":["1752-1947"],"issn-type":[{"value":"1752-1947","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,8,21]]},"assertion":[{"value":"19 May 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 July 2020","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"21 August 2020","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"This study did not include experiments on animals or humans. The patient gave consent to use his details for this case study.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"133"}}