From the Department of Neurology (A.H., J.E.A., J.Y.M., J.H.B.), Mayo Clinic, Rochester, MN; SUNY Downstate College of Medicine (J.M.M.), Brooklyn, NY; University of Pennsylvania (J.R.W.), Philadelphia; and Parkinson's Disease Research, Education and Clinical Center (PADRECC) (J.R.W.), Philadelphia VA Medical Center, PA.
Neurology. 2016 Feb 2;86(5):458-64. doi: 10.1212/WNL.0000000000002328. Epub 2016 Jan 8.
To evaluate the clinical, electrophysiologic, and treatment outcome features of orthostatic tremor (OT) in a large case series.
We performed medical record review of 184 patients who met clinical and electrodiagnostic criteria for OT from 1976 to 2013 at the Mayo Clinic. Demographic, clinical, electrophysiologic, and treatment data were extracted.
The majority of OT cases were female (63.6%) and mean age at onset was 59.3 years (range 13-85 years). Diagnosis was delayed by a mean of 7.2 years (range 0-44 years). The average tremor frequency was 15.7 Hz (range 12.5-20 Hz), and transmitted to the arms on weight-bearing (95.5%). Patients reported a spectrum of progressive orthostatic leg symptoms, relieved by sitting or leaning. Falls were reported in 24.1%. Coexistent neurologic disorders included essential tremor (22.8%), other tremor (4.9%), and parkinsonism (8.7%). Family history of OT was noted in 4.9%. Of 46 medications trialed, 24 failed to provide any benefit. Benzodiazepines provided at least mild benefit in 55.9%, and moderate to marked benefit in 31.5%; β-blockers (31.0%) and anticonvulsants (25.0%) provided mild benefit, and the remainder were largely ineffective. Medication benefit waned over time. Deep brain stimulation (DBS) was effective in 2 cases.
OT predominantly affects female seniors, and the diagnosis should be considered with any orthostatic-induced leg symptoms, and confirmed by surface EMG. Benzodiazepines are the most efficacious treatment, followed by β-blockers and anticonvulsants. DBS should be further explored for treatment.
评估大型病例系列中直立性震颤(OT)的临床、电生理和治疗结果特征。
我们对 1976 年至 2013 年期间在梅奥诊所符合 OT 临床和电诊断标准的 184 名患者进行了病历回顾。提取了人口统计学、临床、电生理和治疗数据。
大多数 OT 病例为女性(63.6%),发病年龄平均为 59.3 岁(范围 13-85 岁)。诊断平均延迟 7.2 年(范围 0-44 年)。平均震颤频率为 15.7 Hz(范围 12.5-20 Hz),承重时会传递到手臂(95.5%)。患者报告了一系列进行性直立性腿部症状,坐或倾斜可缓解。24.1%的患者报告有跌倒。并存的神经障碍包括特发性震颤(22.8%)、其他震颤(4.9%)和帕金森病(8.7%)。OT 家族史占 4.9%。46 种试验药物中有 24 种没有任何益处。苯二氮䓬类药物至少有 55.9%的轻度益处,31.5%的中度至明显益处;β-受体阻滞剂(31.0%)和抗惊厥药(25.0%)有轻度益处,其余药物则基本无效。药物益处随时间推移而减弱。深部脑刺激(DBS)对 2 例有效。
OT 主要影响女性老年人,任何由直立引起的腿部症状都应考虑诊断,并通过表面肌电图确认。苯二氮䓬类药物是最有效的治疗方法,其次是β-受体阻滞剂和抗惊厥药。应进一步探索 DBS 治疗。