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

J Orthop Surg Res. 2022 Jan 15;17(1):19. doi: 10.1186/s13018-021-02905-5.

Abstract

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.

Keywords: Cervical spine; Cross-sectional area; Hirayama disease; Local kyphotic deformity; Posterior cervical extensors.

MeSH terms

  • Adolescent
  • Cervical Vertebrae / diagnostic imaging*
  • Female
  • Humans
  • Kyphosis / diagnostic imaging*
  • Lordosis*
  • Male
  • Retrospective Studies
  • Spinal Muscular Atrophies of Childhood / diagnostic imaging*
  • Treatment Outcome
  • Young Adult

Supplementary concepts

  • Amyotrophy, monomelic