Abstract
Purpose
Hirayama disease, a rare cervical myelopathy in children and young adults, leads to progressive upper limb weakness and muscle loss. Non-invasive external cervical orthosis has been shown to prevent further neurologic decline; however, this treatment modality has not been successful at restoring neurologic and motor function, especially in long standing cases with significant weakness. The pathophysiology remains not entirely understood, complicating standardized operative guidelines; however, some studies report favorable outcomes with internal fixation. We report a successful surgically treated case of pediatric Hirayama disease, supplemented by a systematic review and collation of reported cases in the literature.
Methods
A review of the literature was performed by searching PubMed, Embase, and Web of Science. Full-length articles were included if they reported clinical data regarding the treatment of at least one patient with Hirayama disease and the neurologic outcome of that treatment. Articles were excluded if they did not provide information on treatment outcomes, were abstract-only publications, or were published in languages other than English.
Results
Of the fifteen articles reviewed, 63 patients were described, with 59 undergoing surgery. This encompassed both anterior and posterior spinal procedures and 1 hand tendon transfer. Fifty-five patients, including one from our institution, showed improvement post-treatment. Eleven of these patients were under 18 years old.
Conclusion
Hirayama disease is an infrequent yet impactful cervical myelopathy with limited high-quality evidence available for optimal treatment. The current literature supports surgical decompression and stabilization as promising interventions. However, comprehensive research is crucial for evolving diagnosis and treatment paradigms.
Similar content being viewed by others
Data availability
No datasets were generated or analysed during the current study.
References
Yin B, Liu L, Geng D-Y (2011) Features of Hirayama disease on fully flexed position cervical MRI. J Int Med Res 39(1):222–228. https://doi.org/10.1177/147323001103900124
Kohno M, Takahashi H, Yagishita A, Tanabe H (1998) ‘Disproportion theory’ of the cervical spine and spinal cord in patients with juvenile cervical flexion myelopathy. A study comparing cervical magnetic resonance images with those of normal controls. Surg Neurol 50(5):421–430. https://doi.org/10.1016/s0090-3019(97)00451-5
Hirayama K (2000) Juvenile muscular atrophy of distal upper extremity (Hirayama disease). Intern Med 39(4):283–290. Available: https://doi.org/10.2169/internalmedicine.39.283
Hou C et al (2012) How does the neck flexion affect the cervical MRI features of Hirayama disease? Neurol Sci 33(5):1101–1105. https://doi.org/10.1007/s10072-011-0912-x
Paredes I, Esteban J, Ramos A, Gonzalez P, Rivas JJ (2014) A severe case of Hirayama disease successfully treated by anterior cervical fusion. J Neurosurg Spine 20(2):191–195. https://doi.org/10.3171/2013.10.SPINE13508
Fu Y, Qin W, Sun QL, Fan DS (2016) Investigation of the compliance of cervical collar therapy in 73 patients with Hirayama disease. Zhonghua Yi Xue Za Zhi 96(43):3485–3488. https://doi.org/10.3760/cma.j.issn.0376-2491.2016.43.009
Song J, Cui Z-Y, Chen Z-H, Jiang J-Y (2020) Analysis of the effect of surgical treatment for the patients with Hirayama disease from the perspective of cervical spine sagittal alignment. World Neurosurg 133:e342–e347. https://doi.org/10.1016/j.wneu.2019.09.025
Song J, Wang H-L, Zheng C-J, Jiang J-Y (2017) Risk factors for surgical results of Hirayama disease: a retrospective analysis of a large cohort. World Neurosurg 105:69–77. https://doi.org/10.1016/j.wneu.2017.05.097
Bohara S, Garg K, Mishra S, Tandon V, Chandra PS, Kale SS (2021) Impact of various cervical surgical interventions in patients with Hirayama’s disease-a narrative review and meta-analysis. Neurosurg Rev 44(6):3229–3247. https://doi.org/10.1007/s10143-021-01540-2
Chiba S, Yonekura K, Nonaka M, Imai T, Matumoto H, Wada T (2004) Advanced Hirayama disease with successful improvement of activities of daily living by operative reconstruction. Intern Med 43(1):79–81. https://doi.org/10.2169/internalmedicine.43.79
Luo J et al (2020) Hirayama disease treated by anterior cervical diskectomy and fusion: case report and literature review. World Neurosurg 141:171–174. https://doi.org/10.1016/j.wneu.2020.06.037
Goel A, Dhar A, Shah A (2017) Multilevel spinal stabilization as a treatment for Hirayama disease: report of an experience with five cases. World Neurosurg 99:186–191. https://doi.org/10.1016/j.wneu.2016.11.143
Ito H, Takai K, Taniguchi M (2014) Cervical duraplasty with tenting sutures via laminoplasty for cervical flexion myelopathy in patients with Hirayama disease: successful decompression of a ‘tight dural canal in flexion’ without spinal fusion. J Neurosurg Spine 21(5):743–752. https://doi.org/10.3171/2014.7.SPINE13955
Hirayama K (1959) Juvenile muscular atrophy of unilateral upper extremity : a new clinical entity. Psychiatr Neurol Jpn 61:2190–2198. Available: https://cir.nii.ac.jp/crid/1573668924856807680. Accessed 08 Jul 2023
Pradhan S (2009) Bilaterally symmetric form of Hirayama disease. Neurology 72(24):2083–2089. https://doi.org/10.1212/WNL.0b013e3181aa5364
Agundez M, Rouco I, Barcena J, Mateos B, Barredo J, Zarranz JJ (2015) Hirayama disease: is surgery an option? Neurologia 30(8):502–509. https://doi.org/10.1016/j.nrl.2013.05.005
Sekioka A et al (2021) A 5-year-old boy with acute neurological disorder from anteflexion-induced cervical cord compression after tracheal surgery: radiological findings similar to Hirayama disease. Int J Pediatr Otorhinolaryngol 140:110491. https://doi.org/10.1016/j.ijporl.2020.110491
Cabona C et al (2019) Are basketball players more likely to develop Hirayama disease? J Neurol Sci 400:142–144. https://doi.org/10.1016/j.jns.2019.03.020
Fukutake T (2020) Hirayama disease can be caused by loss of attachment of the cervical posterior Dura to the pedicle due to immunological abnormalities of the Dura and posterior ligaments: a new hypothesis. Brain Nerve 72(12):1371–1381. https://doi.org/10.11477/mf.1416201695
Gamez J et al (2007) Investigation of the role of SMN1 and SMN2 haploinsufficiency as a risk factor for Hirayama’s disease: clinical, neurophysiological and genetic characteristics in a Spanish series of 13 patients. Clin Neurol Neurosurg 109(10):844–848. https://doi.org/10.1016/j.clineuro.2007.07.019
Huang Y-L, Chen C-J (2011) Hirayama disease. Neuroimaging Clin N Am 21(4):939–950. https://doi.org/10.1016/j.nic.2011.07.009
Salome M, Barkhof F, Visser L (2017) Hirayama disease; an atypical clinical manifestation of a cervical myelopathy with typical MRI features. BMJ Case Rep. https://doi.org/10.1136/bcr-2016-217780
Fu Y, Pei X, Zhang J, Kang D, Han H, Fan D (2008) Morphological changes of the lower cervical spinal cord under neutral and fully flexed position by MRI in Chinese patients with Hirayama’s disease. Amyotroph Lateral Scler 9(3):156–162. https://doi.org/10.1080/17482960701726123
Schröder R et al (1999) MRI findings in Hirayama’s disease: flexion-induced cervical myelopathy or intrinsic motor neuron disease? J Neurol 246(11):1069–1074. https://doi.org/10.1007/s004150050514
Huaijun L et al (2006) The diagnosis of a fully flexed neck position MRI diagnosis in juvenile muscular atrophy of the distal upper extremity. Zhonghua Fang She Xue Za Zhi 40(7):745–747. Available: https://inis.iaea.org/search/search.aspx?orig_q=RN:39111200. Accessed 08 Jul 2023
Lai V, Wong YC, Poon WL, Yuen MK, Fu YP, Wong OW (2011) 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 80(3):724–728. https://doi.org/10.1016/j.ejrad.2010.07.021
McGregor S, Joswig H, Duggal N, Miller TA (2017) Hirayama disease: a diagnostic and therapeutic challenge. Can J Neurol Sci 44(6):754–756. https://doi.org/10.1017/cjn.2017.219
Lin M-S, Kung W-M, Chiu W-T, Lyu R-K, Chen C-J, Chen T-Y (2010) Hirayama disease. J Neurosurg Spine 12(6):629–634. https://doi.org/10.3171/2009.12.SPINE09431
Yagi K, Kinoshita K, Hara K, Matsubara S, Uno M (2020) Markedly improved function in severe Hirayama disease by anterior cervical fusion: a case report. Interdiscip Neurosurg 21:100738. https://doi.org/10.1016/j.inat.2020.100738
Xu Q, Gu R, Zhu Q, Suya D (2019) A severe case of Hirayama disease successfully treated by posterior cervical fixation without decompression and fusion. World Neurosurg 122:326–330. https://doi.org/10.1016/j.wneu.2018.10.157
Kuo Y-H, Kuo C-H, Huang W-C, Wu J-C (2019) Anterior cervical discectomy and fusion for Hirayama disease: a case report and literature review. Neurospine 16(3):626–630. https://doi.org/10.14245/ns.1836178.089
Wang H-L et al (2018) Cortical activation changes in Hirayama disease after anterior cervical decompression and fusion. World Neurosurg 116:e588–e594. https://doi.org/10.1016/j.wneu.2018.05.045
Dohzono S, Toyoda H, Tamura A, Hayashi K, Terai H, Nakamura H (2019) Surgical treatment of a patient with prolonged exacerbation of Hirayama disease. Spine Surg Relat Res 3(1):95–97. https://doi.org/10.22603/ssrr.2018-0037
Kikuchi S, Tashiro K, Kitagawa M, Iwasaki Y, Abe H (1987) A mechanism of juvenile muscular atrophy localized in the hand and forearm (Hirayama’s disease)--flexion myelopathy with tight dural canal in flexion. Rinsho Shinkeigaku 27(4):412–419. Available: https://www.ncbi.nlm.nih.gov/pubmed/3621739. Accessed 7 July 2023
Guo X, Lu M, Xie N, Guo Q, Ni B (2014) Multilevel anterior cervical discectomy and fusion with plate fixation for juvenile unilateral muscular atrophy of the distal upper extremity accompanied by cervical kyphosis. J Spinal Disord Tech 27(7):E241–E246. https://doi.org/10.1097/BSD.0000000000000098
Singh R, Hudson M, Meyer JH, Neal MT, Patel NP (2022) Surgical treatment of spinal cord compression due to Hirayama disease: illustrative case. J Neurosur Case Lessons 3(10). https://doi.org/10.3171/CASE21697. Available: https://thejns.org/caselessons/view/journals/j-neurosurg-case-lessons/3/10/article-CASE21697.xml. Accessed 07 Jul 2023
Srivastava SK, Marathe N, Raj A, Bhosale S, Dhole K (2020) Surgical management of Hirayama disease: a rare entity with unusual clinical features. Asian J Neurosurg 15(2):405–408. https://doi.org/10.4103/ajns.AJNS_291_19
Watanabe K, Hasegawa K, Hirano T, Endo N, Yamazaki A, Homma T (2005) Anterior spinal decompression and fusion for cervical flexion myelopathy in young patients. J Neurosurg Spine 3(2):86–91. https://doi.org/10.3171/spi.2005.3.2.0086
Fujimoto Y, Oka S, Tanaka N, Nishikawa K, Kawagoe H, Baba I (2002) Pathophysiology and treatment for cervical flexion myelopathy. Eur Spine J 11(3):276–285. https://doi.org/10.1007/s005860100344
Kohno M, Takahashi H, Ide K, Yamakawa K, Saitoh T, Inoue K (1999) Surgical treatment for patients with cervical flexion myelopathy. J Neurosurg 91(1 Suppl):33–42. https://doi.org/10.3171/spi.1999.91.1.0033
Lu F et al (2013) Efficacy of anterior cervical decompression and fusion procedures for monomelic amyotrophy treatment: a prospective randomized controlled trial: clinical article. J Neurosurg Spine 19(4):412–419. https://doi.org/10.3171/2013.4.SPINE12575
Author information
Authors and Affiliations
Contributions
M.P. and G.R. contributed equally to the manuscript, and should be considered co-first authors, preparing manuscript text and figures, executing PRISMA search, and performing data analysis jointly. V.K. provided additional work on the main manuscript. All authors contributed to the design and conception of the manuscript, providing necessary review.
Corresponding author
Ethics declarations
Conflict of interest
The authors declare no competing interests.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Supplementary Information
Below is the link to the electronic supplementary material.
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
Prablek, M., Reyes, G., Kannan, V. et al. Anterior cervical discectomy and fusion for the treatment of pediatric Hirayama disease. Childs Nerv Syst 40, 1427–1434 (2024). https://doi.org/10.1007/s00381-024-06281-3
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-024-06281-3