Department of Neurology, E-DA Hospital /I-Shou University, Kaohsiung, Taiwan.
Department of Neurology, E-DA Cancer Hospital/I-Shou University, Kaohsiung, Taiwan.
J Formos Med Assoc. 2022 Jul;121(7):1310-1316. doi: 10.1016/j.jfma.2021.09.019. Epub 2021 Oct 14.
BACKGROUND/PURPOSE: Orthostatic myoclonus (OM) is myoclonic jerks in both legs during standing. It may relate to gait unsteadiness and may be associated with various diagnoses, including neurodegenerative, systemic, and musculoskeletal diseases. Diagnosis is based on the surface electromyography (SEMG).
From January 2016 to June 2020, we retrospectively reviewed 35 patients diagnosed with OM based on the SEMG criteria and analyzed the electrophysiological data.
The mean age was 75.3±8.9. Disease duration ranged from 2 days to several years. Genders were equally affected. The most common symptom was gait disturbance, and the next was leg tremulous sensation, followed by backward disequilibrium. 28.6% of our patients had falls. Electrophysiologically, bursting duration shorter than 100 ms supported the myoclonic origin from the cortex. The bursting activity occurred only on the upright and weight-bearing leg. The associated diagnoses included lumbosacral radiculopathy (28.6%), lumbosacral radiculopathy plus myasthenia gravis (2.9%), lumbosacral radiculopathy plus vascular parkinsonism (5.7%), diabetic polyneuropathy (5.7%), vascular parkinsonism (17.1%), Parkinson's disease (PD) (14.2%), normal pressure hydrocephalus (5.7%), medication-induced parkinsonism (2.9%), cervical myelopathy (2.9%), medication-related myoclonus (2.9%), and unknown (11.4%). In PD, OM was present before, along with, or after PD diagnosis. The myoclonic activity disrupted the parkinsonian tremor upon standing on SEMG.
OM is a syndrome instead of a diagnosis. It is more prevalent in the elderly with gait disturbance and patients will not necessarily fall. It is associated with central and peripheral nerve system disorders, systemic diseases, and medication use. We hypothesize that OM is a pathological disintegration of motor circuit.
背景/目的:直立性肌阵挛(OM)是指站立时双腿出现肌阵挛抽搐。它可能与步态不稳有关,可能与各种诊断有关,包括神经退行性、系统性和肌肉骨骼疾病。诊断基于表面肌电图(SEMG)。
从 2016 年 1 月至 2020 年 6 月,我们回顾性分析了 35 例根据 SEMG 标准诊断为 OM 的患者的电生理数据。
平均年龄为 75.3±8.9 岁。疾病持续时间从 2 天到数年不等。男女发病率相当。最常见的症状是步态障碍,其次是腿部震颤感,随后是向后失衡。28.6%的患者有跌倒。电生理上,持续时间短于 100ms 的爆发支持源于皮质的肌阵挛起源。爆发活动仅发生在直立和承重腿上。相关诊断包括腰骶神经根病(28.6%)、腰骶神经根病合并重症肌无力(2.9%)、腰骶神经根病合并血管帕金森病(5.7%)、糖尿病性多发性神经病(5.7%)、血管帕金森病(17.1%)、帕金森病(PD)(14.2%)、正常压力脑积水(5.7%)、药物诱导的帕金森病(2.9%)、颈椎病(2.9%)、药物相关性肌阵挛(2.9%)和未知原因(11.4%)。在 PD 中,OM 出现在 PD 诊断之前、同时或之后。在 SEMG 上,肌阵挛活动打断了站立时的帕金森震颤。
OM 是一种综合征,而不是一种诊断。它在老年患者中更为常见,伴有步态障碍,患者不一定会跌倒。它与中枢和周围神经系统疾病、全身性疾病和药物使用有关。我们假设 OM 是运动回路病理性解体的结果。