{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,20]],"date-time":"2026-02-20T22:15:14Z","timestamp":1771625714794,"version":"3.50.1"},"reference-count":50,"publisher":"Springer Science and Business Media LLC","issue":"10","license":[{"start":{"date-parts":[[2011,6,8]],"date-time":"2011-06-08T00:00:00Z","timestamp":1307491200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nervenarzt"],"published-print":{"date-parts":[[2011,10]]},"DOI":"10.1007\/s00115-011-3320-9","type":"journal-article","created":{"date-parts":[[2011,6,6]],"date-time":"2011-06-06T14:51:33Z","timestamp":1307371893000},"page":"1264-1272","source":"Crossref","is-referenced-by-count":9,"title":["Hirayama-Syndrom in Deutschland","Hirayama disease in Germany"],"prefix":"10.1007","volume":"82","author":[{"given":"J.-S.","family":"Kang","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Jochem-Gawehn","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H.","family":"Laufs","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Ferbert","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P.","family":"Vieregge","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"U.","family":"Ziemann","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2011,6,8]]},"reference":[{"key":"3320_CR1","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1007\/s10072-008-0987-1","volume":"29","author":"Ammendola","year":"2008","unstructured":"Ammendola A, Gallo A, Iannaccone T et al (2008) Hirayama disease: three cases assessed by F wave, somatosensory and motor evoked potentials and magnetic resonance imaging not supporting flexion myelopathy. Neurol Sci 29:303\u2013311","journal-title":"Neurol Sci"},{"key":"3320_CR2","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1097\/NRL.0b013e31818d6717","volume":"15","author":"Andreadou","year":"2009","unstructured":"Andreadou E, Christodoulou K, Manta P et al (2009) Familial asymmetric distal upper limb amyotrophy (Hirayama disease): report of a Greek family. Neurologist 15:156\u2013160","journal-title":"Neurologist"},{"key":"3320_CR3","doi-asserted-by":"crossref","unstructured":"Arrese I, Rivas JJ, Esteban J et al (2009) A case of Hirayama disease treated with laminectomy and duraplasty without spinal fusion. Neurocirugia (Asturias, Spain) 20:555\u2013558","DOI":"10.1016\/S1130-1473(09)70134-4"},{"key":"3320_CR4","doi-asserted-by":"crossref","first-page":"1426","DOI":"10.1212\/01.WNL.0000120665.81001.40","volume":"62","author":"Baba","year":"2004","unstructured":"Baba Y, Nakajima M, Utsunomiya H et al (2004) Magnetic resonance imaging of thoracic epidural venous dilation in Hirayama disease. Neurology 62:1426\u20131428","journal-title":"Neurology"},{"key":"3320_CR5","doi-asserted-by":"crossref","first-page":"237","DOI":"10.3109\/17482960902849823","volume":"11","author":"Blumen","year":"2010","unstructured":"Blumen SC, Drory VE, Sadeh M et al (2010) Mutational analysis of glycyl-tRNA synthetase (GARS) gene in Hirayama disease. Amyotroph Lateral Scler 11:237\u2013239","journal-title":"Amyotroph Lateral Scler"},{"key":"3320_CR6","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1148\/radiol.2311030004","volume":"231","author":"Chen","year":"2004","unstructured":"Chen CJ, Hsu HL, Tseng YC et al (2004) Hirayama flexion myelopathy: neutral-position MR imaging findings \u2013 importance of loss of attachment. Radiology 231:39\u201344","journal-title":"Radiology"},{"key":"3320_CR7","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1177\/0883073809336876","volume":"25","author":"Chen","year":"2010","unstructured":"Chen TH, Hung CH, Hsieh TJ et al (2010) Symmetric atrophy of bilateral distal upper extremities and hyperIgEaemia in a male adolescent with Hirayama disease. J Child Neurol 25:371\u2013374","journal-title":"J Child Neurol"},{"key":"3320_CR8","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1007\/s10072-010-0405-3","volume":"31","author":"Ciceri","year":"2010","unstructured":"Ciceri EF, Chiapparini L, Erbetta A et al (2010) Angiographically proven cervical venous engorgement: a possible concurrent cause in the pathophysiology of Hirayama\u2019s myelopathy. Neurol Sci 31:845\u2013848","journal-title":"Neurol Sci"},{"key":"3320_CR9","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1001\/archneurpsyc.1947.02300330110009","volume":"58","author":"Drooz","year":"1947","unstructured":"Drooz RB (1947) Benign focal amyotrophy. Arch Neurol Psychiatry 58:498\u2013505","journal-title":"Arch Neurol Psychiatry"},{"key":"3320_CR10","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1002\/mus.21307","volume":"40","author":"Elsheikh","year":"2009","unstructured":"Elsheikh B, Kissel JT, Christoforidis G et al (2009) Spinal angiography and epidural venography in juvenile muscular atrophy of the distal arm \u201eHirayama disease\u201c. Muscle Nerve 40:206\u2013212","journal-title":"Muscle Nerve"},{"key":"3320_CR11","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1007\/s005860100344","volume":"11","author":"Fujimoto","year":"2002","unstructured":"Fujimoto Y, Oka S, Tanaka N et al (2002) Pathophysiology and treatment for cervical flexion myelopathy. Eur Spine J 11:276\u2013285","journal-title":"Eur Spine J"},{"key":"3320_CR12","doi-asserted-by":"crossref","first-page":"844","DOI":"10.1016\/j.clineuro.2007.07.019","volume":"109","author":"Gamez","year":"2007","unstructured":"Gamez J, Also E, Alias L et al (2007) Investigation of the role of SMN1 and SMN2 haploinsufficiency as a risk factor for Hirayama\u2019s disease: clinical, neurophysiological and genetic characteristics in a Spanish series of 13 patients. Clin Neurol Neurosurg 109:844\u2013848","journal-title":"Clin Neurol Neurosurg"},{"key":"3320_CR13","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1034\/j.1600-0404.2003.02142.x","volume":"107","author":"Gouri-Devi","year":"2003","unstructured":"Gouri-Devi M, Nalini A (2003) Long-term follow-up of 44 patients with brachial monomelic amyotrophy. Acta Neurol Scand 107:215\u2013220","journal-title":"Acta Neurol Scand"},{"key":"3320_CR14","doi-asserted-by":"crossref","first-page":"388","DOI":"10.1001\/archneur.1984.04050160050015","volume":"41","author":"Gouri-Devi","year":"1984","unstructured":"Gouri-Devi M, Suresh TG, Shankar SK (1984) Monomelic amyotrophy. Arch Neurol 41:388\u2013394","journal-title":"Arch Neurol"},{"key":"3320_CR15","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1016\/0887-8994(91)90089-4","volume":"7","author":"G\u00fcc\u00fcyener","year":"1991","unstructured":"G\u00fcc\u00fcyener K, Aysun S, Topaloglu H et al (1991) Monomelic amyotrophy in siblings. Pediatr Neurol 7:220\u2013222","journal-title":"Pediatr Neurol"},{"key":"3320_CR16","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/0022-510X(96)00154-2","volume":"141","author":"Guglielmo","year":"1996","unstructured":"Guglielmo GD, Brahe C, Di Muzio A et al (1996) Benign monomelic amyotrophies of upper and lower limb are not associated to deletions of survival motor neuron gene. J Neurol Sci 141:111\u2013113","journal-title":"J Neurol Sci"},{"key":"3320_CR17","first-page":"283","volume":"Med","author":"Hirayama","year":"2000","unstructured":"Hirayama K (2000) Juvenile muscular atrophy of distal upper extremity (Hirayama disease). Intern Med (Tokyo) 39:283\u2013290","journal-title":"Intern"},{"key":"3320_CR18","first-page":"2190","volume":"61","author":"Hirayama","year":"1959","unstructured":"Hirayama K (1959) Juvenile muscular atrophy of unilateral upper extremity a new clinical entity. Psychiatr Neurol Jpn 61:2190\u20132197","journal-title":"Psychiatr Neurol Jpn"},{"key":"3320_CR19","doi-asserted-by":"crossref","first-page":"1922","DOI":"10.1212\/WNL.54.10.1922","volume":"54","author":"Hirayama","year":"2000","unstructured":"Hirayama K, Tokumaru Y (2000) Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity. Neurology 54:1922\u20131926","journal-title":"Neurology"},{"key":"3320_CR20","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1136\/jnnp.50.3.285","volume":"50","author":"Hirayama","year":"1987","unstructured":"Hirayama K, Tomonaga M, Kitano K et al (1987) Focal cervical poliopathy causing juvenile muscular atrophy of distal upper extremity: a pathological study. J Neurol Neurosurg Psychiatry 50:285\u2013290","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"3320_CR21","doi-asserted-by":"crossref","first-page":"576","DOI":"10.1002\/mus.20980","volume":"37","author":"Huang","year":"2008","unstructured":"Huang YC, Ro LS, Chang HS et al (2008) A clinical study of Hirayama disease in Taiwan. Muscle Nerve 37:576\u2013582","journal-title":"Muscle Nerve"},{"key":"3320_CR22","first-page":"11","volume":"40","author":"Imai","year":"2000","unstructured":"Imai T, Shizukawa H, Nakanishi K et al (2000) Hyperexcitability of cervical motor neurons during neck flexion in patients with Hirayama disease. Electromyogr Clin Neurophysiol 40:11\u201315","journal-title":"Electromyogr Clin Neurophysiol"},{"key":"3320_CR23","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1136\/jnnp.2003.031609","volume":"76","author":"Ito","year":"2005","unstructured":"Ito S, Kuwabara S, Fukutake T et al (2005) HyperIgEaemia in patients with juvenile muscular atrophy of the distal upper extremity (Hirayama disease). J Neurol Neurosurg Psychiatry 76:132\u2013134","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"3320_CR24","doi-asserted-by":"crossref","first-page":"935","DOI":"10.3171\/jns.1987.66.6.0935","volume":"66","author":"Iwasaki","year":"1987","unstructured":"Iwasaki Y, Tashiro K, Kikuchi S et al (1987) Cervical flexion myelopathy: a \u201etight dural canal mechanism\u201c. Case report. J Neurosurg 66:935\u2013937","journal-title":"J Neurosurg"},{"key":"3320_CR25","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1097\/NRL.0b013e3181942880","volume":"15","author":"Kajikawa","year":"2009","unstructured":"Kajikawa H, Kokubo Y, Taniguchi A et al (2009) Juvenile muscular atrophy of the distal upper extremity (hirayama disease) in two lanky look-alike brothers. Neurologist 15:220\u2013222","journal-title":"Neurologist"},{"key":"3320_CR26","unstructured":"Kiguchi S, Tashiro K (2003) Juvenile muscular atrophy of distal upper extremity (Hirayama\u2019s disease). In: Jones HR, De Vivo DC, Darras BT (Hrsg) Neuromuscular disorders of infancy, childhood, and adolescence: a clinician\u2019s approach. Butterworth Heinemann, Philadelphia, S 167\u2013181"},{"key":"3320_CR27","doi-asserted-by":"crossref","first-page":"798","DOI":"10.1136\/jnnp.70.6.798","volume":"70","author":"Kira","year":"2001","unstructured":"Kira J, Ochi H (2001) Juvenile muscular atrophy of the distal upper limb (Hirayama disease) associated with atopy. J Neurol Neurosurg Psychiatry 70:798\u2013801","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"3320_CR28","first-page":"33","volume":"91","author":"Kohno","year":"1999","unstructured":"Kohno M, Takahashi H, Ide K et al (1999) Surgical treatment for patients with cervical flexion myelopathy. J Neurosurg 91:33\u201342","journal-title":"J Neurosurg"},{"key":"3320_CR29","doi-asserted-by":"crossref","unstructured":"Konno S, Goto S, Murakami M et al (1997) Juvenile amyotrophy of the distal upper extremity: pathologic findings of the dura mater and surgical management. Spine (Phila Pa 1976) 22:486\u2013492","DOI":"10.1097\/00007632-199703010-00004"},{"key":"3320_CR30","doi-asserted-by":"crossref","unstructured":"Lai V, Wong YC, Poon Wl et al (2010) Forward shifting of posterior dural sac during flexion cervical magnetic resonance imaging in Hirayama disease: An initial study on normal subjects compared to patients with Hirayama disease. Eur J Radiol [Epub ahead of print]","DOI":"10.1016\/j.ejrad.2010.07.021"},{"key":"3320_CR31","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1016\/0924-980X(94)00246-4","volume":"97","author":"Misra","year":"1995","unstructured":"Misra UK, Kalita J (1995) Central motor conduction in Hirayama disease. Electroencephalogr Clin Neurophysiol 97:73\u201376","journal-title":"Electroencephalogr Clin Neurophysiol"},{"key":"3320_CR32","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1001\/archneur.62.1.120","volume":"62","author":"Misra","year":"2005","unstructured":"Misra UK, Kalita J, Mishra VN et al (2005) A clinical, magnetic resonance imaging, and survival motor neuron gene deletion study of Hirayama disease. Arch Neurol 62:120\u2013123","journal-title":"Arch Neurol"},{"key":"3320_CR33","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1136\/jnnp.2005.082362","volume":"77","author":"Misra","year":"2006","unstructured":"Misra UK, Kalita J, Mishra VN et al (2006) Effect of neck flexion on F wave, somatosensory evoked potentials, and magnetic resonance imaging in Hirayama disease. J Neurol Neurosurg Psychiatry 77:695\u2013698","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"3320_CR34","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1016\/j.jns.2004.02.015","volume":"220","author":"Nalini","year":"2004","unstructured":"Nalini A, Lokesh L, Ratnavalli E (2004) Familial monomelic amyotrophy a case report from India. J Neurol Sci 220:95\u201398","journal-title":"J Neurol Sci"},{"key":"3320_CR35","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1016\/S0022-510X(02)00277-0","volume":"206","author":"Ochi","year":"2003","unstructured":"Ochi H, Murai H, Osoegawa M et al (2003) Juvenile muscular atrophy of distal upper extremity associated with airway allergy: two cases successfully treated by plasma exchange. J Neurol Sci 206:109\u2013114","journal-title":"J Neurol Sci"},{"key":"3320_CR36","doi-asserted-by":"crossref","first-page":"E1196","DOI":"10.1227\/01.NEU.0000345951.24132.7F","volume":"64","author":"Patel","year":"2009","unstructured":"Patel TR, Chiocca EA, Freimer ML et al (2009) Lack of epidural pressure change with neck flexion in a patient with Hirayama disease: case report. Neurosurgery 64:E1196\u2013E1197","journal-title":"Neurosurgery"},{"key":"3320_CR37","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1136\/jnnp.74.5.627","volume":"74","author":"Polo","year":"2003","unstructured":"Polo A, Curro\u2018 Dossi M, Fiaschi A et al (2003) Peripheral and segmental spinal abnormalities of median and ulnar somatosensory evoked potentials in Hirayama\u2019s disease. J Neurol Neurosurg Psychiatry 74:627\u2013632","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"3320_CR38","doi-asserted-by":"crossref","first-page":"2083","DOI":"10.1212\/WNL.0b013e3181aa5364","volume":"72","author":"Pradhan","year":"2009","unstructured":"Pradhan S (2009) Bilaterally symmetric form of Hirayama disease. Neurology 72:2083\u20132089","journal-title":"Neurology"},{"key":"3320_CR39","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/S0022-510X(96)00296-1","volume":"146","author":"Pradhan","year":"1997","unstructured":"Pradhan S, Gupta RK (1997) Magnetic resonance imaging in juvenile asymmetric segmental spinal muscular atrophy. J Neurol Sci 146:133\u2013138","journal-title":"J Neurol Sci"},{"key":"3320_CR40","doi-asserted-by":"crossref","first-page":"1980","DOI":"10.1212\/01.WNL.0000068017.75780.44","volume":"60","author":"Restuccia","year":"2003","unstructured":"Restuccia D, Rubino M, Valeriani M et al (2003) Cervical cord dysfunction during neck flexion in Hirayama\u2019s disease. Neurology 60:1980\u20131983","journal-title":"Neurology"},{"key":"3320_CR41","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1001\/archneur.1997.00550130032012","volume":"54","author":"Robberecht","year":"1997","unstructured":"Robberecht W, Aguirre T, Van Den Bosch L et al (1997) Familial juvenile focal amyotrophy of the upper extremity (Hirayama disease). Superoxide dismutase 1 genotype and activity. Arch Neurol 54:46\u201350","journal-title":"Arch Neurol"},{"key":"3320_CR42","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1016\/0022-510X(87)90169-9","volume":"80","author":"Schlegel","year":"1987","unstructured":"Schlegel U, Jerusalem F, Tackmann W et al (1987) Benign juvenile focal muscular atrophy of upper extremities \u2013 a familial case. J Neurol Sci 80:351\u2013353","journal-title":"J Neurol Sci"},{"key":"3320_CR43","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1016\/j.jns.2008.07.020","volume":"275","author":"Tanaka","year":"2008","unstructured":"Tanaka M, Ishizu T, Ochi H et al (2008) Intrathecal upregulation of IFN-gamma and MIP-1beta in juvenile muscular atrophy of the distal upper extremity. J Neurol Sci 275:74\u201377","journal-title":"J Neurol Sci"},{"key":"3320_CR44","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1080\/14660820500396877","volume":"7","author":"Tashiro","year":"2006","unstructured":"Tashiro K, Kikuchi S, Itoyama Y et al (2006) Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan. Amyotroph Lateral Scler 7:38\u201345","journal-title":"Amyotroph Lateral Scler"},{"key":"3320_CR45","first-page":"173","volume":"Neurol","author":"Tokumaru","year":"2001","unstructured":"Tokumaru Y, Hirayama K (2001) Cervical collar therapy for juvenile muscular atrophy of distal upper extremity (Hirayama disease) results from 38 cases. Clin Neurol (Rinsho shinkeigaku) 41:173\u2013178","journal-title":"Clin"},{"key":"3320_CR46","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1136\/jnnp.58.1.56","volume":"58","author":"Toma","year":"1995","unstructured":"Toma S, Shiozawa Z (1995) Amyotrophic cervical myelopathy in adolescence. J Neurol Neurosurg Psychiatry 58:56\u201364","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"3320_CR47","doi-asserted-by":"crossref","first-page":"1036","DOI":"10.1093\/brain\/awg117","volume":"126","author":"Van","year":"2003","unstructured":"Van Den Berg-Vos RM, Visser J, Franssen H et al (2003) Sporadic lower motor neuron disease with adult onset: classification of subtypes. Brain 126:1036\u20131047","journal-title":"Brain"},{"key":"3320_CR48","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1034\/j.1600-0404.2001.00074.x","volume":"104","author":"Willeit","year":"2001","unstructured":"Willeit J, Kiechl S, Kiechl-Kohlendorfer U et al (2001) Juvenile asymmetric segmental spinal muscular atrophy (Hirayama\u2019s disease) three cases without evidence of \u201eflexion myelopathy\u201c. Acta Neurol Scand 104:320\u2013322","journal-title":"Acta Neurol Scand"},{"key":"3320_CR49","first-page":"511","volume":"Med","author":"Yaguchi","year":"2007","unstructured":"Yaguchi H, Takahashi I, Tashiro J et al (2007) Scapular winging as a symptom of cervical flexion myelopathy. Intern Med (Tokyo) 46:511\u2013513","journal-title":"Intern"},{"key":"3320_CR50","doi-asserted-by":"crossref","first-page":"133","DOI":"10.3109\/17482960902912407","volume":"11","author":"Zhou","year":"2010","unstructured":"Zhou B, Chen L, Fan D et al (2010) Clinical features of Hirayama disease in mainland China. Amyotroph Lateral Scler 11:133\u2013139","journal-title":"Amyotroph Lateral Scler"}],"container-title":["Der Nervenarzt"],"original-title":[],"language":"de","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00115-011-3320-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s00115-011-3320-9\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00115-011-3320-9","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,6,11]],"date-time":"2019-06-11T12:55:01Z","timestamp":1560257701000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s00115-011-3320-9"}},"subtitle":["Fallberichte und Literatur\u00fcbersicht","Case reports and review of the literature"],"short-title":[],"issued":{"date-parts":[[2011,6,8]]},"references-count":50,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2011,10]]}},"alternative-id":["3320"],"URL":"https:\/\/doi.org\/10.1007\/s00115-011-3320-9","relation":{},"ISSN":["0028-2804","1433-0407"],"issn-type":[{"value":"0028-2804","type":"print"},{"value":"1433-0407","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,6,8]]}}}