Singhal Somya, Mahajan Sanyam K, Jha Sanjeev, Singh Vivek, Mani Vinita E, Paliwal Vimal K
Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
Department of Radiology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
Ann Indian Acad Neurol. 2025 Jul 1;28(4):527-534. doi: 10.4103/aian.aian_8_25. Epub 2025 Aug 7.
To correlate the distribution of neurogenic motor unit potentials in the upper limb (s) and the extent of anterior displacement of the cervical duramater on neck flexion with the progression of weakness/atrophy in Hirayama disease.
Consecutive patients with distal Hirayama disease were classified as distal group (neurogenic potential in C7-T1 innervated muscles), proximal (neurogenic potentials C5-T1 muscles), and contralateral group (neurogenic potentials in contralateral hand/arm). Based on the extent of anterior dural displacement on neck-flexed cervical magnetic resonance imaging, patients were classified as anterior dural displacement across the C5 vertebra and anterior dural displacement at C5 vertebra and below. The disease progression at 1 year was correlated with the distribution of neurogenic potentials and the extent of anterior dural displacement.
Twenty-eight patients (mean age, 17.41 ± 2.30 years; all males) were included. Eleven (39.2%), 17 (60.7%), and 22 (78%) patients were in proximal, distal, and contralateral groups, respectively. Twenty-three (82%) had anterior dural displacement across the C5 vertebra, whereas 5 (17%) had anterior dural displacement at C5 vertebra and below. Ipsilateral disease progression was seen in 15 (53%) and contralateral progression in 25 (89%) (new onset in 7 [25%]). No patient showed progression in shoulder/arm muscles. The proximal group had a significantly larger extent of anterior dural displacement. However, there was no correlation of disease progression with either the distribution of neurogenic motor unit potentials or the extent of cervical dural displacement on neck flexion.
The extent of anterior dural displacement on neck flexion and neurogenic motor unit potentials in proximal, distal, or contralateral upper limb did not correlate with progression of muscle weakness/atrophy in Hirayama disease at 1 year.
探讨上肢神经源性运动单位电位的分布以及颈部屈曲时颈段硬脊膜向前移位的程度与平山病肌无力/萎缩进展的相关性。
将连续性远端平山病患者分为远端组（C7 - T1 节段支配肌肉出现神经源性电位）、近端组（C5 - T1 节段肌肉出现神经源性电位）和对侧组（对侧手/臂出现神经源性电位）。根据颈部屈曲位颈椎磁共振成像上硬脊膜向前移位的程度，将患者分为硬脊膜向前移位超过 C5 椎体以及硬脊膜向前移位在 C5 椎体及以下。将 1 年时的疾病进展与神经源性电位的分布以及硬脊膜向前移位的程度进行相关性分析。
共纳入 28 例患者（平均年龄 17.41 ± 2.30 岁；均为男性）。分别有 11 例（39.2%）、17 例（60.7%）和 22 例（78%）患者属于近端组、远端组和对侧组。23 例（82%）患者硬脊膜向前移位超过 C5 椎体，而 5 例（17%）患者硬脊膜向前移位在 C5 椎体及以下。同侧疾病进展见于 15 例（53%），对侧进展见于 25 例（89%）（7 例[25%]为新发）。无患者肩部/臂部肌肉出现进展。近端组硬脊膜向前移位的程度显著更大。然而，疾病进展与神经源性运动单位电位的分布以及颈部屈曲时颈段硬脊膜移位的程度均无相关性。
颈部屈曲时硬脊膜向前移位的程度以及近端、远端或对侧上肢的神经源性运动单位电位与平山病 1 年时肌无力/萎缩的进展无关。
