Is there cervical spine muscle weakness in patients with Hirayama disease? A morphological study about cross-sectional areas of muscles on MRI

Li, Zhechen; Zhang, Wenming; Wu, Wence; Wei, Chao; Chen, Xuanwei; Lin, Jianhua

doi:10.1007/s00586-020-06290-1

Cited by 8 publications

(16 citation statements)

References 26 publications

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“…Li et al found that the imbalance between the strengths of flexor and extensor muscles that was found in HD patients during cervical flexion resulted in dynamic instability and showed that it was a risk factor for spinal cord atrophy. However, all of the above findings need to be confirmed in future studies (50). Wang et al found that the neutral and flexion Cobb angles in HD patients decreased from C2/3 to C5/6 but increased at C6/7; this may be another cause of the observed dynamic instability (51).…”

Section: Pathogenesismentioning

confidence: 89%

Update on the Pathogenesis, Clinical Diagnosis, and Treatment of Hirayama Disease

Wang

Tian

et al. 2022

Front. Neurol.

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Hirayama disease (HD) is characterized by the juvenile onset of unilateral or asymmetric weakness and amyotrophy of the hand and ulnar forearm and is most common in males in Asia. A perception of compliance with previous standards of diagnosis and treatment appears to be challenged, so the review is to update on HD. First, based on existing theory, the factors related to HD includes, (1) cervical cord compression during cervical flexion, (2) immunological factors, and (3) other musculoskeletal dynamic factors. Then, we review the clinical manifestations: typically, (1) distal weakness and wasting in one or both upper extremities, (2) insidious onset and initial progression for 3–5 years, (3) coarse tremors in the fingers, (4) cold paralysis, and (5) absence of objective sensory loss; and atypically, (1) positive pyramidal signs, (2) atrophy of the muscles of the proximal upper extremity, (3) long progression, and (4) sensory deficits. Next, updated manifestations of imaging are reviewed, (1) asymmetric spinal cord flattening, and localized lower cervical spinal cord atrophy, (2) loss of attachment between the posterior dural sac and the subjacent lamina, (3) forward displacement of the posterior wall of the cervical dural sac, (4) intramedullary high signal intensity in the anterior horn cells on T2-weighted imaging, and (5) straight alignment or kyphosis of cervical spine. Thus, the main manifestations of eletrophysiological examinations in HD include segmental neurogenic damages of anterior horn cells or anterior roots of the spinal nerve located in the lower cervical spinal cord, without disorder of the sensory nerves. In addition, definite HD needs three-dimensional diagnostic framework above, while probable HD needs to exclude other diseases via “clinical manifestations” and “electrophysiological examinations”. Finally, the main purpose of treatment is to avoid neck flexion. Cervical collar is the first-line treatment for HD, while several surgical methods are available and have achieved satisfactory results. This review aimed to improve the awareness of HD in clinicians to enable early diagnosis and treatment, which will enable patients to achieve a better prognosis.

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Section: Pathogenesismentioning

confidence: 89%

Update on the Pathogenesis, Clinical Diagnosis, and Treatment of Hirayama Disease

Wang

Tian

et al. 2022

Front. Neurol.

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“…PCEs weakness is a cause of adolescent idiopathic kyphosis [ 38 ]. Extensor muscle strength is weaker than flexor muscle strength in people with poor cervical curvature, as has been reported for HD [ 24 ]. The cervical spine is associated with PCEs, similar to “bow” and “string.” Numerous studies have highlighted the importance of strengthening muscles for spinal sagittal balance [ 11 , 28 , 39 ].…”

Section: Discussionmentioning

confidence: 65%

“…Cervical muscle strength is lower in HD patients than in normal people, and there is a mismatch between flexor muscles and extensor muscles [ 24 ]. Changes in extensor muscle strength may affect cervical sagittal alignment.…”

Section: Discussionmentioning

confidence: 99%

“…Most of the vertebrae involved in kyphosis are C 3 –C 6 , and PCEs atrophy can cause abnormal cervical curvature, especially of the extensor. In HD patients, cervical muscle CSA is smaller than in normal people, and there is flexor and extensor muscle mismatch [ 24 ]. We speculate that atrophy of the cervical posterior extensor muscle in HD is a reason for abnormal cervical sagittal alignment.…”

Section: Discussionmentioning

confidence: 99%

“…Muscle cross-sectional area was measured by two independent spine surgeons (Table 1 ), Tian and Xie, using the ImageJ (National Institutes of Health, America) to measure the ROI (Fig. 1 ) on the CSA of the PCEs based on the techniques used in previous studies [ 24 ]. Selected images were all T2-weighted magnetic resonance images (MRIs) parallel to the C 5 –C 6 intervertebral disk axis MR. PCEs include deep extensors (DEs) and superficial extensors (SEs).…”

Section: Methodsmentioning

confidence: 99%

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Why the patients with Hirayama disease have abnormal cervical sagittal alignment? A radiological measurement analysis of posterior cervical extensors

et al. 2022

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Purpose To explore the relationship between the strength of posterior cervical extensors (PCEs) and cervical sagittal alignment in Hirayama disease (HD) patients. Methods We analyzed the (magnetic resonance imaging) MRI T2WI and X-rays of 60 HD patients who visited Huashan Hospital from June 2017 to February 2020. Symptoms of these patients include adolescent onset, manifestation of unilateral upper limb muscle weakness and muscle atrophy of the forearm and hand. MRI images were used to measure (the cross-sectional area) CSA of cervical PCEs. The ratio of muscle CSA to vertebral body areas at the same level is defined as R-CSA. Cervical sagittal alignment includes the C2–7 Cobb angle, T1 slope and C2–7 sagittal vertical axis (SVA). The geometric center of the C3–6 vertebral body was determined using the line connecting the C2 inferior endplate and the C7 upper endplate. When located behind the line, it is defined as a “local kyphotic deformity.” The number of vertebral bodies involved in kyphotic deformity was determined by measuring the local kyphosis angle (LKA). Spearman correlation analysis (α = 0.05) was used to determine the relationship between R-CSA and sagittal parameters. ROC curves were used to analyze the sensitivity and specificity of relevant variables. Results Spearman correlation test revealed that R-CSA negatively correlated with T1S (S = 0.34, r = 0.34, p = 0.01) and LKA (S = 0.44, r = 0.5, p = 0.01), but did not correlate with the C2-C7 Cobb angle (S = 0.20, p = 0.12) or C2-C7 SVA (S = − 0.17, p = 0.46). (p < 0.05). ROC curve analysis showed that the areas under the curve (AUCs) of the T1 slope and LKA was 0.6696 and 0.7646, respectively. T1 slope, cutoff value: 17.2°; sensitivity: 0.5806; specificity: 0.7241; p < 0.05. LKA: cutoff value: − 14°; sensitivity: 1; specificity: 0.5333; p < 0.05. Conclusions In patients with Hirayama disease, the strength of posterior cervical extensors and cervical sagittal alignment are closely related. The local kyphosis angle can be used as a reference for the strength of posterior cervical extensors. These results indicate the weakness of PCEs, which may predispose the cervical spine of HD patients to a less stable situation. Therefore, patients with Hirayama disease should strengthen the exercise of the PCEs.

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Efficacy of Anterior Cervical Discectomy and Fusion for Female Patients with Hirayama Disease

Wang¹,

Tian²,

Wu³

et al. 2023

World Neurosurgery

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Is there cervical spine muscle weakness in patients with Hirayama disease? A morphological study about cross-sectional areas of muscles on MRI

Cited by 8 publications

References 26 publications

Update on the Pathogenesis, Clinical Diagnosis, and Treatment of Hirayama Disease

Update on the Pathogenesis, Clinical Diagnosis, and Treatment of Hirayama Disease

Why the patients with Hirayama disease have abnormal cervical sagittal alignment? A radiological measurement analysis of posterior cervical extensors

Efficacy of Anterior Cervical Discectomy and Fusion for Female Patients with Hirayama Disease

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