Ferri R, Musumeci S A, Elia M, Del Gracco S, Scuderi C, Bergonzi P
Oasi Institute for Research on Mental Retardation and Brain Aging (IRCCS), Troina, Italy.
Neurophysiol Clin. 1994 Dec;24(6):413-26. doi: 10.1016/s0987-7053(05)80074-7.
Middle-latency somatosensory evoked potentials (MLSEPs) were recorded from 19 scalp electrodes in ten male patients with the fragile X (fraX) syndrome and nine normal controls. One fraX patient was found presenting the so-called "giant" MLSEPs with an amplitude of N60 of about 60 microV and of 40 microV after stimulation of the right and left median nerves, respectively. Tapping of the right hand, in the same patient, induced the appearance of left parietal evoked EEG spikes. These findings further support the already suggested similarity between the epileptic picture of several fraX patients with that of the benign childhood epilepsy with centrotemporal spikes. Color mapping of the MLSEPs recorded in the remaining nine patients, when compared with the control group, showed an abnormally large N30 over the frontal regions, together with an increase in amplitude of P27, over the parietal areas, and of N60 and P100 which also presented abnormal field distributions, being represented preferentially over the frontal regions. These data could suggest the existence of a cortical dysfunction mostly involving the frontal lobes (supplementary motor area, in particular) in the fraX syndrome which could support many behavioral changes usually observed in these patients.
对10名患有脆性X综合征（fraX）的男性患者和9名正常对照者，从19个头皮电极记录了中潜伏期体感诱发电位（MLSEPs）。发现1名fraX患者呈现所谓的“巨大”MLSEPs，分别刺激右侧和左侧正中神经后，N60波幅约为60微伏和40微伏。在同一名患者中，轻敲右手诱发了左侧顶叶诱发脑电图棘波的出现。这些发现进一步支持了已经提出的几种fraX患者的癫痫表现与伴有中央颞区棘波的良性儿童癫痫之间的相似性。与对照组相比，对其余9名患者记录的MLSEPs进行彩色映射显示，额叶区域的N30异常增大，顶叶区域的P27波幅增加，N60和P100的波幅也增加，且其场分布异常，主要出现在额叶区域。这些数据可能表明在fraX综合征中存在主要累及额叶（特别是辅助运动区）的皮质功能障碍，这可能支持这些患者通常观察到的许多行为变化。
