Niedermeyer E, Naidu S B
The Johns Hopkins Hospital, Baltimore, Maryland 21287, USA.
Eur Child Adolesc Psychiatry. 1998 Jun;7(2):69-72. doi: 10.1007/s007870050049.
EEG data in Rett syndrome (RS) are quite often characterized by spikes over the central region (especially in sleep). Furthermore, there has been a recent observation of a prominent and peculiar theta (4-6/s) rhythm over the central region and vicinity. In view of the clinical phenomena of RS, it is pointed out that clinical and electrical findings are based upon hyperexcitability of the motor cortex (or a state of motor cortex dyscontrol). States of dyscontrol (disinhibition) are caused by deficits in higher cortical functions. It is reasonable to assume a primary dysfunction for the frontal lobe which is also smaller in size than other cerebral regions. The behavioral (motor) disturbance in RS can be easily explained as the expression of disturbed prefrontal/premotor function with special effect on the "syntax of action". It is further theorized that such a state of fronto-motor cortex uncoupling may also exist in attention deficit hyperkinetic disorder (ADHD) even though there is hardly any structural CNS impairment in that condition. A presumed "lazy frontal lobe" is likely to react favorably to the stimulation provided by methylphenidate (a therapeutic effect which should not be viewed as paradoxical).
雷特综合征（RS）的脑电图数据通常以中央区棘波为特征（尤其是在睡眠中）。此外，最近还观察到中央区及其附近有明显且特殊的θ波（4 - 6次/秒）节律。鉴于RS的临床现象，有人指出临床和电生理发现是基于运动皮层的过度兴奋（或运动皮层失控状态）。失控状态（去抑制）是由高级皮层功能缺陷引起的。假设额叶存在原发性功能障碍是合理的，因为额叶在大小上也比其他脑区小。RS中的行为（运动）障碍很容易被解释为前额叶/运动前区功能紊乱的表现，对“动作语法”有特殊影响。进一步推测，即使注意力缺陷多动障碍（ADHD）几乎没有任何中枢神经系统结构损伤，这种额-运动皮层解耦状态也可能存在。推测的“懒惰额叶”可能对哌甲酯提供的刺激有良好反应（这种治疗效果不应被视为自相矛盾）。
