Pralong E, Pollo C, Coubes P, Bloch J, Roulet E, Tétreault M-H, Debatisse D, Villemure J-G
UNN-NS BH 13, CHUV, 1011 Lausanne, Switzerland.
Neurophysiol Clin. 2005 Nov-Dec;35(5-6):168-73. doi: 10.1016/j.neucli.2005.12.004. Epub 2006 Jan 18.
Lesch-Nyhan syndrome is a rare and debilitating condition characterized by dystonia and self-mutilating behavior. In order to shed light on the pathophysiology of dystonia, we report the pallidal electrophysiological activity recorded in two patients during deep brain stimulation surgery (DBS).
Microrecordings were performed on 162 neurons along four tracks aimed at the right and left anterior (limbic) and posterior (motor) globus pallidus internus (GPI).
Regardless of the anesthetic agent used (propofol or sevoflurane), both patients showed similar neurons firing rates in the four regions studied, namely the limbic and motor portions of the globus pallidus externus (GPE) or GPI. In both patients, firing rates were similar in the GPE (12.2+/-1.8 Hz, N=38) and GPI (13.2+/-1.0 Hz, N=83) portions of the limbic track, while the motor GPE fired at a higher frequency (23.8+/-2.7 Hz, N=18) than the motor GPI (12.5+/-1.4 Hz, N=23).
These results demonstrate that light propofol or sevoflurane anesthesia influences pallidal activity in a similar way. Electrophysiological recordings suggest that Lesch-Nyhan syndrome might be characterized by analogous firing frequencies in the limbic GPE and GPI while motor GPE would tend to fire at higher rate than the motor GPI. It is therefore tempting to suggest that the symptoms that are observed in Lesch-Nyhan syndrome might result from motor GPI inhibition.
This observation may confirm the Albin and Delong's model of the basal nuclei in hypokinetic and hyperkinetic disorders.
莱施-奈恩综合征是一种罕见且使人衰弱的疾病，其特征为肌张力障碍和自残行为。为了阐明肌张力障碍的病理生理学机制，我们报告了在两名患者进行脑深部电刺激手术（DBS）期间记录到的苍白球电生理活动。
沿着针对左右前（边缘）和后（运动）内侧苍白球（GPI）的四条轨迹对162个神经元进行了微记录。
无论使用何种麻醉剂（丙泊酚或七氟醚），两名患者在所研究的四个区域，即外侧苍白球（GPE）或GPI的边缘和运动部分，均显示出相似的神经元放电频率。在两名患者中，边缘轨迹的GPE（12.2±1.8Hz，N = 38）和GPI（13.2±1.0Hz，N = 83）部分的放电频率相似，而运动GPE的放电频率（23.8±2.7Hz，N = 18）高于运动GPI（12.5±1.4Hz，N = 23）。
这些结果表明，轻度丙泊酚或七氟醚麻醉以相似的方式影响苍白球活动。电生理记录表明，莱施-奈恩综合征的特征可能是边缘GPE和GPI具有相似的放电频率，而运动GPE的放电频率往往高于运动GPI。因此，很容易推测莱施-奈恩综合征中观察到的症状可能是由于运动GPI受抑制所致。
这一观察结果可能证实了阿尔宾和德隆关于基底核在运动减少和运动增多性疾病中的模型。
