European Spine Journal (2018) 27:1207–1208 https://doi.org/10.1007/s00586-018-5610-4
REVIEWER’S COMMENT
Expert’s Comment concerning Grand Rounds case entitled “Hirayama disease” by D. C. Kieser, P. J. Cox, S. C. J. Kieser (Eur Spine J; 2018: doi:10.1007/s00586‑018‑5545‑9) Takeo Furuya1 Received: 2 April 2018 / Accepted: 12 April 2018 / Published online: 20 April 2018 © Springer-Verlag GmbH Germany, part of Springer Nature 2018
This is a well-written case report and literature review describing Hirayama disease in a 21-year-old man with progressive weakness of the hand and forearm [1]. The paper highlights many of the important aspects of performing the diagnosis by neurological examination and imaging studies. It shows a representative photographic image of the forearms of the patient and describes the characteristic sign of Hirayama disease, which is an oblique border of muscular atrophy on both the volar and dorsal forearms, a finding widely known as ‘oblique atrophy’. The paper also describes the importance of dynamic imaging followed by MRI with flexion of the cervical spine and CT myelography with the neck in the neutral position and flexion position. I will not discuss the many specific points mentioned in the "Grand Rounds" portion of the paper, except to make specific comments about the treatment of Hirayama disease. Conservative therapy using the soft collar is emphasized in
* Takeo Furuya furuya‑takeo@chiba‑u.jp 1
this paper. As the authors discussed in the paper, there is no doubt that conservative management is the first-line treatment of choice, and that it has shown excellent results with a successful arrest of disease progression in many patients and occasional reversal of signs and symptoms. I would like to add information regarding the paper [2] that the authors cited (reference 5 in this report). Tashiro et al. reported the positive effects of cervical collar immobilization. They also reported the efficacy of surgical treatment with various surgical techniques in 41 cases to restrict the forward flexion of the cervical spine or to permit the dura mater to relax. They concluded that all these techniques showed favorable therapeutic effects. The discussion of the surgical indications and the surgical outcomes is limited in the paper. The efficacy of surgical treatment has been reported in previous articles [3–9]. The purpose of surgical intervention is to prevent the progression of neurological signs and symptoms. Readers should be informed that there are patients with this disease who progress to irreversible deficits. Because of such cases, we should bear in mind the limitations of conservative treatment and consider performing surgical treatment and discontinuing conservative therapy. This case study and Grand Rounds underscores the importance of differential diagnosis in young patients with muscular weakness and atrophy of muscles of the forearms. It contributes to closing the gap between our current level of knowledge and a comprehensive understanding of Hirayama disease.
Compliance with ethical standards Conflict of interest The author declares that he has no conflict of interest.
Spine Division, Department of Orthopaedics, Chiba University Graduate School of Medicine, 1‑8‑1 Inohana, Chuo‑ku, Chiba 260‑8670, Japan
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References 1. Kieser DC, Cox PJ, Kieser SCJ (2018) Hirayama disease. Eur Spine J. https://doi.org/10.1007/s00586-018-5545-9 2. Tashiro K, Kikuchi S, Itoyama Y, Tokumaru Y, Sobue G, Mukai E, Akiguchi I, Nakashima K, Kira J, Hirayama K (2006) Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan. Amyotroph Lateral Scler 7(1):38–45 3. Lin MS, Kung WM, Chiu WT, Lyu RK, Chen CJ, Chen TY (2010) Hirayama disease. J Neurosurg Spine 12(6):629–634 4. Lu F, Wang H, Jiang J, Chen W, Ma X, Ma X, Xia X, Wang L (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 5. 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
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European Spine Journal (2018) 27:1207–1208 6. 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 7. 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 8. 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 9. Fujimori T, Tamura A, Miwa T, Iwasaki M, Oda T (2017) Severe cervical flexion myelopathy with long tract signs: a case report and a review of literature. Spinal Cord Ser Cases 11(3):17016
