Posterior cervical fixation versus long-term cervical collar for management of Hirayama Disease: Prospective randomized open blinded endpoint (PROBE), phase III study

1.      Research Hypothesis:The surgical intervention is superior to neck immobilization using a cervical collar in Hirayama disease in terms of clinical and radiological improvement at six months after surgery.

2.      Background and Rationale of the StudyHirayama disease (HD), also known as juvenile muscular atrophy, or monomelic amyotrophy (MMA) affecting the upper extremity, is a special neurological disorder first brought to attention by a Japanese neurologist, Keizo Hirayama in 1959.(5) In India, similar cases, as well as variants, have been published (6). The disease predominantly affects males in their second decade (3) and leads to considerable morbidity in their economically productive stage of life. The long-term follow-up studies of HD have shown that the patients progressed for 4-5 years before stabilization of the disease but with significant residual neurological deficits.(7) The putative etiology described is dynamic compression of the cervical dural canal in flexed neck posture causing dynamic compression and ischemia of lower cervical cord (5), while post-infectious (3) and genetic (8,9) etiologies have also been postulated.

Although several surgical interventions are described (4), there is a glaring evidence-practice gap in management. The evidence of surgical benefit is limited to case series(10–12), or case-control studies. (2) In the modified Delphi technique to understand the diagnosis and treatment of HD, 40.5% of experts opted for surgical treatment when the disease duration is less than 4 years and there has been disease progression in the past 6 months, while 38.1% opted for conservative treatment and 21.4% were neutral.(13) A recent case-control study (2) found 76% in the surgical group had clinical improvement while none in the conservative group. This huge effect size should be exploited to benefit young patients, by generating a level I evidence.

The rationale of surgical intervention in patients with HD is to immobilize the spine in extension to prevent compression over the cervical spinal cord, which occurs in flexion. The use of several surgical approaches in patients with HD have been described in the literature. The rate of neurological improvement has been found to be similar between anterior and posterior surgical approaches in a recent meta-analysis.(4) It has shown that posterior cervical fixation carries the least risk of blood loss. Hence, we have chosen this surgical approach.

References:

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2.         Thakar S, Arun AA, Rajagopal N, Aryan S, Mohan D, Vijayan JE, et al. Outcomes after Cervical Duraplasty for Monomelic Amyotrophy (Hirayama Disease): Results of a Case–Control Study of 60 Patients. J Neurosci Rural Pract. 2021 Sep 22;12(4):642–51.

3.         Vibha D, Behari M, Goyal V, Shukla G, Bhatia R, Srivastava AK, et al. Clinical profile of Monomelic Amyotrophy (MMA) and role of persistent viral infection. J Neurol Sci. 2015 Dec 15;359(1–2):4–7.

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5.         Hirayama K, Tomonaga M, Kitano K, Yamada T, Kojima S, Arai K. Focal cervical poliopathy causing juvenile muscular atrophy of distal upper extremity: a pathological study. J Neurol Neurosurg Psychiatry. 1987 Mar 1;50(3):285–90.

6.         Gourie-Devi M, Suresh TG. Madras pattern of motor neuron disease in South India. J Neurol Neurosurg Psychiatry. 1988 Jun;51(6):773–7.

7.         A N, M GD, K T, Ah R. Monomelic amyotrophy: clinical profile and natural history of 279 cases seen over 35 years (1976-2010). Amyotroph Lateral Scler Front Degener [Internet]. 2014 Sep [cited 2022 Mar 14];15(5–6). Available from: https://pubmed.ncbi.nlm.nih.gov/24853410/

8.         Atchayaram N, Vasudev MK, Goel G. Familial monomelic amyotrophy (Hirayama disease): two brothers with classical flexion induced dynamic changes of the cervical dural sac. Neurol India. 2009 Dec;57(6):810–2.

9.         Lim YM, Koh I, Park YM, Kim JJ, Kim DS, Kim HJ, et al. Exome sequencing identifies KIAA1377 and C5orf42 as susceptibility genes for monomelic amyotrophy. Neuromuscul Disord NMD. 2012 May;22(5):394–400.

10.       Lu F, Wang H, Jiang J, Chen W, Ma X, Ma X, et al. Efficacy of anterior cervical decompression and fusion procedures for monomelic amyotrophy treatment: a prospective randomized controlled trial: Clinical article. J Neurosurg Spine. 2013 Oct 1;19(4):412–9.

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13.       Lyu F, Zheng C, Wang H, Nie C, Ma X, Xia X, et al. Establishment of a clinician-led guideline on the diagnosis and treatment of Hirayama disease using a modified Delphi technique. Clin Neurophysiol Off J Int Fed Clin Neurophysiol. 2020 Jun;131(6):1311–9.

14.       Broekema AEH, Molenberg R, Kuijlen JMA, Groen RJM, Reneman MF, Soer R. The Odom Criteria: Validated at Last: A Clinimetric Evaluation in Cervical Spine Surgery. J Bone Joint Surg Am. 2019 Jul 17;101(14):1301–8.

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