Department of Neurology and Clinical Neurophysiology, Academic Medical Center, P.O. Box 22660, 1100 DD Amsterdam, The Netherlands.
J Neurol. 2010 Aug;257(8):1349-55. doi: 10.1007/s00415-010-5531-6. Epub 2010 Mar 30.
Propriospinal myoclonus (PSM) is a rare disorder with repetitive flexor, arrhythmic jerks of the trunk, hips and knees. Its generation is presumed to relay in the spinal cord. We report a case series of 35 consecutive patients with jerks of the trunk referred as possible PSM to a tertiary referral center for movement disorders. We review classical PSM features as well as psychogenic and tic characteristics. In our case series, secondary PSM was diagnosed in one patient only. 34 patients showed features suggestive of a psychogenic origin of axial jerks. Diagnosis of psychogenic axial jerks was based on clinical clues without additional investigations (n = 8), inconsistent findings at polymyography (n = 15), regular eye blinking preceding jerks (n = 2), or the presence of a Bereitschaftspotential (BP) (n = 9). In addition, several tic characteristics were noted. Almost all patients referred with possible PSM in our tertiary referral clinic had characteristics suggesting a psychogenic origin even in the presence of a classic polymyography pattern or in the absence of a BP. Clinical overlap with adult-onset tics seems to exist.
propriospinal 肌阵挛(PSM)是一种罕见的疾病,表现为躯干、臀部和膝盖的反复屈肌、不规则的抽搐。其产生被认为与脊髓有关。我们报告了一组 35 例连续的因可能的 PSM 而转诊至运动障碍三级转诊中心的患者。我们回顾了经典的 PSM 特征以及心因性和抽搐特征。在我们的病例系列中,仅诊断出 1 例继发性 PSM。34 例患者表现出提示轴向抽搐为心因性起源的特征。心因性轴向抽搐的诊断基于临床线索,无需额外检查(n = 8)、肌电图检查结果不一致(n = 15)、抽搐前有规律的眨眼(n = 2)或存在准备电位(BP)(n = 9)。此外,还注意到了一些抽搐特征。几乎所有在我们的三级转诊诊所因可能的 PSM 而转诊的患者都具有心因性起源的特征,即使存在典型的肌电图模式或不存在 BP 也是如此。与成人起病的抽动似乎存在临床重叠。