Radiological and Electrophysiological Correlates of Disease Progression in Patients with Hirayama Disease.

IF 1.8 4区医学 Q3 CLINICAL NEUROLOGY

Annals of Indian Academy of Neurology Pub Date : 2025-07-01 Epub Date: 2025-08-07 DOI:10.4103/aian.aian_8_25

Somya Singhal, Sanyam K Mahajan, Sanjeev Jha, Vivek Singh, Vinita E Mani, Vimal K Paliwal

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Abstract

Background and objectives: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

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平山病患者疾病进展的放射学和电生理学相关性。

背景和目的：探讨平山病中上肢神经源性运动单位电位的分布和颈部屈曲时颈硬膜前移位的程度与无力/萎缩的进展之间的关系。方法：将平山病患者连续分为远端组（C7-T1神经支配肌的神经源性电位）、近端组（C5-T1神经源性电位）和对侧组（对侧手/手臂的神经源性电位）。根据颈屈曲颈磁共振成像硬脑膜前移位的程度，将患者分为横跨C5椎体的硬脑膜前移位和C5椎体及以下的硬脑膜前移位。1年的疾病进展与神经源性电位的分布和硬脑膜前部移位的程度有关。结果：28例患者，平均年龄17.41±2.30岁；所有男性均包括在内。近端组11例（39.2%），远端组17例（60.7%），对侧组22例（78%）。23例（82%）在C5椎体有前硬膜移位，而5例（17%）在C5椎体及以下有前硬膜移位。同侧疾病进展15例（53%），对侧疾病进展25例（89%）（新发7例[25%]）。没有患者表现出肩部/手臂肌肉的进展。近端组硬脑膜前部移位程度明显较大。然而，疾病进展与神经源性运动单位电位的分布或颈部屈曲时颈硬膜移位的程度没有相关性。结论：1年后平山病患者颈部屈曲的前硬脑膜移位程度和近端、远端或对侧上肢的神经源性运动单位电位与肌肉无力/萎缩的进展无关。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Annals of Indian Academy of Neurology Nervous System Diseases-

CiteScore

2.20

自引率

11.80%

发文量

293

审稿时长

29 weeks

期刊介绍： The journal has a clinical foundation and has been utilized most by clinical neurologists for improving the practice of neurology. While the focus is on neurology in India, the journal publishes manuscripts of high value from all parts of the world. Journal publishes reviews of various types, original articles, short communications, interesting images and case reports. The journal respects the scientific submission of its authors and believes in following an expeditious double-blind peer review process and endeavors to complete the review process within scheduled time frame. A significant effort from the author and the journal perhaps enables to strike an equilibrium to meet the professional expectations of the peers in the world of scientific publication. AIAN believes in safeguarding the privacy rights of human subjects. In order to comply with it, the journal instructs all authors when uploading the manuscript to also add the ethical clearance (human/animals)/ informed consent of subject in the manuscript. This applies to the study/case report that involves animal/human subjects/human specimens e.g. extracted tooth part/soft tissue for biopsy/in vitro analysis.