儿童起病与成人起病肌张力障碍苍白球内侧神经波动比较及临床意义

Comparison of oscillatory activity in globus pallidus internus between childhood-onset and adult-onset dystonia and its clinical significance

目的比较儿童起病与成人起病的肌张力障碍的苍白球内侧(GPi)神经波动特点。方法对8例儿童期起病原发性肌张力障碍(儿童起病组)与10例成人期起病的原发性肌张力障碍患者(成人起病组)行脑深部电刺激术(DBS),均以双侧GPi为靶点。记录患者坐姿静息态和不自主运动状态下局部场电位及受累肌肉肌电信号,对局部场电位进行功率谱分析和统计学检验。结果标准化功率谱密度分析显示肌张力障碍在苍白球内侧部所记录的神经波动于Theta、Alpha低频段的能量明显增强。儿童起病组肌张力障碍的不自主运动状态在4～10 Hz频段能量成分较静息态升高,在10～25 Hz、27～38 Hz频段能量成分较静息态下降;与静息态比较,不自主运动状态14～16.5 Hz、20～22 Hz频段能量下降,且差异有统计学意义(均P<0.05)。成人起病组的肌张力障碍的不自主运动状态在4～6 Hz频段能量成分较静息态显著升高(P<0.05),在6.5～20 Hz频段能量成分较静息态下降,而在9～11 Hz、13～14.5 Hz频段有显著差异(均P<0.05)。与成人起病组比较,儿童起病组肌张力障碍不自主运动状态在13.5～16 Hz的低Beta频段能量显著降低(P<0.05),在高Beta 29～37 Hz频段的能量同步化更显著(P<0.05)。在静息态时,儿童起病组肌张力障碍静息态时在7.5～9.5 Hz、13～14.5 Hz频段的能量同步化较成人起病组更低(均P<0.05),而在30～38Hz多个频段(30～32.5 Hz,33.5～35 Hz,36.5～38Hz)的能量同步化较成人起病肌张力障碍更明显(均P<0.05)。结论进一步证明肌张力障碍存在同步化的低频能量特征(约4～10 Hz)。儿童起病肌张力障碍在高Beta频段的能量同步化更显著,成人起病肌张力障碍在Theta、Alpha低频的能量同步化更明显。提示不同类型的肌张力障碍表现出不同的神经波动特征,DBS治疗时可能需要不同的刺激策略。

Objective Comparison of the oscillatory activity in globus pallidus internus（GPi） between childhood-onset and adult-onset dystonia. Methods Eight patients with childhood-onset dystonia and ten patients with adult-onset dystonia underwent DBS electrode implantation targeting the GPi. The local field potentials and muscle EMG signals under resting and involuntary movements were recorded and power spectrum analysis and statistical tests were performed on the local field potentials. Results Normalized power spectra of GPi local field potentials significantly enhanced in theta and alpha frequency band in dystonia patients. In childhood-onset group,the state of involuntary movement has higher power over the 4-10 Hz frequency band than the resting state,while it has lower power than the resting state over the 10-25 Hz and 27-38 Hz frequency bands. There is a significance over 14-16. 5 Hz and 20-22 Hz（P 〈 0. 05）. In adult-onset group,involuntary movement had higher power over the 4-6 Hz than the resting state,while it has lower power than the resting state over the 6. 5-20 Hz. There is a significance over 9-11 Hz and 13-4. 5 Hz（P 〈 0. 05）. In voluntary movement,childhood-onset dystonia patients had higher beta band（29-37 Hz） and lower beta band（13. 5-16 Hz） compared with adult-onset dystonia（P 〈0. 05）. In resting,childhood-onset dystonia patients had lower power over low frequency bands（7. 5-9. 5 Hz,13-14. 5 Hz） and higher power over 30-38 Hz（30-32. 5 Hz,33. 5-35 Hz,36. 5-38 Hz） compared with adultonset dystonia（P 〈 0. 0 5）. Conclusions This result provides further study that dystonia is characterized by lowfrequency（4-10 Hz）. And the result suggests that childhood-onset dystonia has higher high beta power than adultonset dystonia,while adult-onset dystonia has higher power over theta、alpha low frequency band. It suggested that different types of dystonia exhibit different characteristics of oscillation activities,and may require different stimulation strategies for treatment.