A Severe Case of Hirayama Disease Successfully Treated by Posterior Cervical Fixation without Decompression and Fusion

doi:10.1016/j.wneu.2018.10.157

World Neurosurgery

Volume 122, February 2019, Pages 326-330

https://doi.org/10.1016/j.wneu.2018.10.157 Get rights and content

Background

Hirayama disease (HD) is a rare disease traditionally treated with nonsurgical procedures. Surgical treatment, which is performed by decompression and/or fusion of the cervical spine, is generally considered for rapidly progressive or advanced cases, and the choice of surgical technique is debatable. We describe the first severe case of HD that was successfully treated by posterior lateral mass screw fixation without decompression or fusion.

Case Description

The patient was a 17-year-old adolescent boy who presented with progressive symptoms bilaterally with an unsatisfactory history of conservative treatment. We performed posterior lateral mass screw fixation without decompression or fusion and removed the internal fixation after 4 years of follow-up. Symptom remission and imaging performance improvement were achieved, and the stability and range of motion of the cervical spine were maintained.

Conclusions

Nondecompression and nonfusion surgery was successful and achieved satisfactory results in this case; therefore, it serves as a promising candidate for the surgical treatment of HD.

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)

Surgical Management of Hirayama Disease (Monomelic Amyotrophy): Systematic Review and Meta-Analysis of Patient-Level Data
2023, World Neurosurgery
Hirayama disease or juvenile-onset monomelic amyotrophy is a clinical syndrome that disproportionately affects young males. Standard of care revolves around conservative management, but some patients experience disease progression that may benefit from surgical intervention.
Using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, a systematic review of previous reports of surgical treatment for Hirayama disease was performed. Studies were included if they provided individual patient-level data, described the clinical presentation and surgical intervention, and reported neurological improvement at last follow-up. Comparison between those who improved and those with stable symptoms at last follow-up was performed. Decision-tree analysis was used to identify the best predictors of neurological improvement by last follow-up.
Of 624 unique articles, 30 were included in the qualitative review and 23 in the meta-analysis. Among the 70 patients in the meta-analysis, mean age was 21.2 ± 6.3 years, 91% were male, and mean symptom duration at presentation was 43.3 ± 61.8 months. Fifty-nine patients (84.3%) had improvement in their neurological symptoms by last follow-up. Univariable analysis showed the only significant predictor of improvement in neurological symptoms by last follow-up was the use of stabilization-alone versus decompression with or without stabilization. Baseline clinical symptoms nor radiographic features predicted outcome. Decision-tree analysis showed surgical strategy (stabilization-alone vs. decompression ± stabilization), age (<20 vs. ≥20), and surgical approach (anterior-only vs. posterior-only or anterior-posterior) predicted a higher likelihood of neurological improvement by last follow-up.
Nearly 85% of patients experienced improvement in neurological symptoms. Improvement was best for those who underwent stabilization-alone, and decision-tree analysis suggested that the likelihood of improvement was also superior for patients under 20 years of age and those treated with an anterior versus posterior or staged approach.
Efficacy of Anterior Cervical Discectomy and Fusion for Female Patients with Hirayama Disease
2023, World Neurosurgery
Citation 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
To compare the preoperative and postoperative hand function and radiographic parameters in female patients with Hirayama disease (HD).
Consecutive female patients with HD undergoing anterior cervical discectomy and fusion were followed up. The postoperative hand functional data were obtained from the last follow-up, whereas the postoperative radiographic data were obtained from the examinations in 3- or 6-month follow-up after surgical treatments. The preoperative and postoperative data of hand functional and radiographic assessments were collected and compared between them. Logistic regression analysis was used to clear potential risk factors for surgical treatment.
In all, 15 female patients with HD were included in the follow-up study over 9 years. Significant differences were found in total scores (P < 0.001) and all 6 dimensions, including function (P = 0.003), activities of daily life (P = 0.002), work (P = 0.003), satisfaction (P = 0.002), appearance (P = 0.005), and HD-specific hand symptoms (P = 0.001) in hand functional assessment. The comparison of C2–C7 Cobb angle was statistically different (P = 0.042) in radiographic assessments. The course of illness was of marginal significance (P = 0.065) with curative effect of surgical treatment in logistic regression analysis.
Anterior cervical discectomy and fusion is an effective way to treat female patients with HD, and the course of illness may be correlated with the efficacy of surgery. For some female patients with HD with a clear diagnosis, early surgical treatment is worthy of clinical consideration.
Markedly improved function in severe Hirayama disease by anterior cervical fusion: A case report
2020, Interdisciplinary Neurosurgery: Advanced Techniques and Case Management
Citation 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.
Hirayama disease (HD) is a juvenile muscular atrophy of the distal upper extremities and is considered to be associated with cervical flexion. HD is managed by conservative treatment using cervical collar therapy or surgery, which may shorten the period of the disease or improve the functional outcome. However, surgical treatment for advanced and severe HD has rarely been reported, and it is unknown whether the surgical treatment effectively improves it. We present a rare case of rapidly progressing and advanced HD that showed remarkable recovery after early surgery.
An 18-year-old man presented with a 4-month history of HD, which rapidly progressed. The patient had severe weakness accompanied by atrophy of the right hand and forearm and mild weakness in the left-side counterparts. Five months after the onset, the patient underwent anterior cervical fixation at C5–7 and showed excellent recovery from severe HD; at postoperative month 8, he had no difficulty in daily life functioning, although a slight muscle weakness remained in the right forearm and hand.
Even in cases of advanced HD, early surgical treatment may be effective in improving the function of upper extremities and could be considered for the treatment of patients.
Anterior cervical discectomy and fusion for the treatment of pediatric Hirayama disease
2024, Child's Nervous System
Anterior cervical discectomy and fixation fusion for Hirayama disease in a young male: Case report
2023, Indian Spine Journal
Surgical treatment of spinal cord compression due to Hirayama disease: illustrative case
2022, Journal of Neurosurgery: Case Lessons

View all citing articles on Scopus

: 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.

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Case ReportA Severe Case of Hirayama Disease Successfully Treated by Posterior Cervical Fixation without Decompression and Fusion

Background

Case Description

Conclusions

Introduction

Section snippets

Case Description

Discussion

Conclusions

Neurochirurgie

Indian J Med Specialities

Surg Neurol

World Neurosurg

World Neurosurg

World Neurosurg

Juvenile muscular atrophy of unilateral upper extremity

Neurology

Juvenile muscular atrophy of unilateral upper extremity: a new clinical entity

Psychiatr Neurol Jpn

Hirayama disease: a rare disease with unusual features

Case Rep Neurol Med

Juvenile Muscular atrophy of distal upper extremity (Hirayama disease)

Intern Med

A confirmed case in the United Kingdom of Hirayama disease in a young white male presenting with hand weakness

World Neurosurg

Case Report
A Severe Case of Hirayama Disease Successfully Treated by Posterior Cervical Fixation without Decompression and Fusion