Hassan Anhar, Mateen Farrah J, Coon Elizabeth A, Ahlskog J Eric
Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Arch Neurol. 2012 Aug;69(8):1032-8. doi: 10.1001/archneurol.2012.161.
To better characterize the clinical features, electrophysiologic features, and treatment outcomes of painful legs and moving toes (PLMT) syndrome.
Large case series.
Neurology outpatient clinic at a tertiary referral center, 1983-2011.
All cases of PLMT seen at our institution during an 18-year period were identified using our medical record linkage system.
Key demographic, clinical, imaging, and electrophysiologic features of PLMT. Treatment outcomes and long-term follow-up are also reported.
Of 76 cases identified (including 50 women [66%]), the mean age at onset was 58 years (range, 24-86 years) and at neurologic evaluation was 63 years (range, 26-88 years). Pure lower limb involvement was most common (69 patients [91%]), and 44 cases (58%) were bilateral. The most frequently diagnosed causes were peripheral neuropathy (21 cases [28%]), previous trauma (8 [11%]), and radiculopathy (7 [9%]); 32 cases (42%) were cryptogenic. Electromyography consistently showed irregular 50-millisecond to 1-second bursts of normal motor unit potential firing at 2 to 200 Hz accompanying the movements. Pain occurred first in nearly all cases and was more distressing to patients than the movements. Both components were difficult to treat, with no consistent benefit from a variety of drugs and therapeutic modalities. The syndrome persisted in most patients (83%) during the mean follow-up of 4.6 years, suggesting low likelihood of spontaneous resolution.
Painful legs and moving toes syndrome is a debilitating clinical syndrome, not because of the movements but rather because of the pain, which often is refractory to treatment. Segmental lower limb involvement is most common, and neurophysiologic findings support a pathophysiologic process localizing to a central generator at the spinal cord or brainstem level.
更好地描述疼痛性腿部与活动脚趾(PLMT)综合征的临床特征、电生理特征及治疗效果。
大型病例系列研究。
1983 - 2011年,一家三级转诊中心的神经科门诊。
利用我们的病历关联系统识别出在18年期间于我院就诊的所有PLMT病例。
PLMT的关键人口统计学、临床、影像学及电生理特征。还报告了治疗效果及长期随访情况。
共识别出76例(包括50名女性[66%]),发病时的平均年龄为58岁(范围24 - 86岁),神经科评估时的平均年龄为63岁(范围26 - 88岁)。单纯下肢受累最为常见(69例[91%]),44例(58%)为双侧受累。最常诊断出的病因是周围神经病变(21例[28%])、既往创伤(8例[11%])和神经根病(7例[9%]);32例(42%)病因不明。肌电图始终显示,随着运动出现正常运动单位电位在2至200赫兹下以50毫秒至1秒的不规则爆发。几乎所有病例中疼痛均先出现,且相较于活动,患者对疼痛更为苦恼。这两个症状均难以治疗,多种药物及治疗方式均未取得一致效果。在平均4.6年的随访期间,大多数患者(83%)的该综合征持续存在,提示自发缓解的可能性较低。
疼痛性腿部与活动脚趾综合征是一种使人衰弱的临床综合征,其导致衰弱的原因并非活动,而是疼痛,且疼痛通常难以治疗。节段性下肢受累最为常见,神经生理学发现支持一种定位于脊髓或脑干水平的中枢发生器的病理生理过程。