Case ReportA Severe Case of Hirayama Disease Successfully Treated by Posterior Cervical Fixation without Decompression and Fusion
Introduction
Initially reported in 1959 and named after its founder, Hirayama disease (HD) is also known as juvenile amyotrophy of the distal upper extremity or cervical flexion myelopathy, with the characteristic of having a benign and self-limiting process.1, 2 With less than 1000 reported cases in the literature, this rare disease is most prevalent among young Asian men (male/female ratio, 20:1)3, 4; however, sporadic cases have been reported in Europe, North America, and Australia.5
The disease is clinically featured by unilateral or bilateral asymmetrical amyotrophy of the forearms and hands with weakness. The pathogenesis of HD is still uncertain, but it is mainly thought to be directly caused by chronic repetitive compression of the lower level spinal cord when the neck is moving to a flexed position.6 Its diagnosis is mainly based on clinical outcomes and flexion magnetic resonance imaging (MRI) findings of the cervical spine, which reveal antedisplacement of the posterior dural sac with spinal cord compression and an enlarged posterior epidural space with engorged epidural veins.7 An early diagnosis followed by a strict cervical collar application to restrict neck flexion is effective in preventing the progression of muscular weakness in the early disease stage.8 Recently, some researchers tentatively treated the disease with surgical procedures. Surgical treatment is generally considered for rapidly progressive or advanced cases, and the choice of surgical technique is debatable.9
We describe the first severe case of HD that was successfully treated by posterior lateral mass screw fixation without decompression or fusion.
Section snippets
Case Description
A 17-year-old adolescent boy visited our clinic with complaints of a 2-year history of insidious, ongoing weakness of the left distal upper limb with progressive left forearm and hand atrophy. He denied any history of trauma, pain, numbness, fever, neck ache, myalgia, and paresthesia. His family history was inconsistent with any neuromuscular disease.
He was initially diagnosed as having HD and received cervical collar treatment elsewhere 2 years before visiting our clinic. Unfortunately, he did
Discussion
Classified as a benign variant of motor neuron disorders or a segmental anterior horn cell disease affecting C5-T1 myotomes, HD mainly affects male adolescents in Asia in their teens and early 20s.4 With an insidious onset, the disease has a static course after 2–6 years of progression, causing lower motor neuron type of weakness affecting the upper limbs unilaterally or bilaterally (asymmetrically).10 In recent years, an increasing number of cases has been reported worldwide, enabling a better
Conclusions
To our knowledge, we are the first to apply the therapeutic concept of nondecompression and nonfusion surgery to treat HD. This attempt was successful and achieved satisfactory results; therefore, nondecompression and nonfusion surgery is a promising candidate for surgical treatment of HD. A longer-term follow-up is needed to further investigate this topic.
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Cited by (10)
Efficacy of Anterior Cervical Discectomy and Fusion for Female Patients with Hirayama Disease
2023, World NeurosurgeryCitation Excerpt :Various surgical approaches have been used in the treatment of HD. Posterior cervical facet fixation in multisegments by the transarticular technique,26 cervical laminectomy and microresection of the posterior venous plexus,3 posterior cervical fixation without decompression and fusion,27 posterior lateral mass instrumentation without fusion,28 anterior cervical corpectomy and fusion,29 and ACDF30 have been successfully performed in clinical practices. However, a meta-analysis showed that although these surgical methods can be used, the most efficient approach is still uncertain.31
Markedly improved function in severe Hirayama disease by anterior cervical fusion: A case report
2020, Interdisciplinary Neurosurgery: Advanced Techniques and Case ManagementCitation Excerpt :However, our patient refused to undergo long-term collar therapy. Various surgical treatments have been reported to effectively arrest the progression of HD and alleviate the patient’s symptoms [3,12–15]. However, there is no general consensus regarding the application of surgical treatment to HD, because HD is a self-limiting disease and its progression stops spontaneously within several years.
Anterior cervical discectomy and fusion for the treatment of pediatric Hirayama disease
2024, Child's Nervous SystemAnterior cervical discectomy and fixation fusion for Hirayama disease in a young male: Case report
2023, Indian Spine JournalSurgical treatment of spinal cord compression due to Hirayama disease: illustrative case
2022, Journal of Neurosurgery: Case Lessons
Conflict of interest statement: This study was supported by the crosswise tasks of Shanghai Sanyou Medical Co.,Ltd. (3R215P403430) in providing the cost of imaging examination for the patient.