Chiron C, Raynaud C, Jambaqué I, Dulac O, Zilbovicius M, Syrota A
Clinique de Pédiatrie et de Puériculture, INSERM U 29, Hôpital Saint Vincent de Paul, Paris, France.
Epilepsy Res. 1991 Apr;8(3):232-40. doi: 10.1016/0920-1211(91)90069-r.
Hemimegalencephaly (HME) is a severe unilateral brain malformation the prognosis of which may be improved by hemispherectomy. HME also provides a unique opportunity to compare normal and pathological hemispheric function in the same patient. We performed a serial functional cerebral imaging study in a child suffering from a neuropathologically confirmed left HME. He was hemispherectomized at 11 months because of intractable epilepsy; this led to cessation of seizures and dramatic psychomotor improvement. Regional cerebral blood flow (rCBF) was studied at 1, 7, 10, 12 and 25 months with single photon computed tomography (SPECT) using 133-Xenon and with simultaneous EEG recording. At one month of age SPECT was performed ictally. During left EEG discharges, rCBF was 40% higher on the left hemisphere than on the right, even in occipital and frontal regions, usually immature at this age. A crossed cerebellar hyperperfusion was also found. At 7 and 10 months, SPECT was performed interictally; rCBF was 45% lower in the left hemisphere than in the right. During follow-up, global and regional CBF values showed normal levels and normal maturation in the right hemisphere except for a mild and transient decrease observed one month after hemispherectomy. SPECT provides an additional procedure for studying hemispheric function in vivo. Serial SPECT imaging may be useful for the preoperative and postoperative evaluation in unilateral cerebral malformation.
半侧巨脑症（HME）是一种严重的单侧脑畸形，半球切除术可能会改善其预后。HME也为比较同一患者正常和病理半球功能提供了独特的机会。我们对一名经神经病理学确诊为左侧HME的儿童进行了系列功能性脑成像研究。由于难治性癫痫，他在11个月大时接受了半球切除术；这导致癫痫发作停止，精神运动能力显著改善。在1、7、10、12和25个月时，使用133-氙单光子计算机断层扫描（SPECT）并同步记录脑电图，研究局部脑血流量（rCBF）。在1个月大时，进行了发作期SPECT检查。在左侧脑电图放电期间，即使在枕叶和额叶区域（通常在这个年龄还未发育成熟），左侧半球的rCBF也比右侧高40%。还发现了交叉性小脑血流灌注过多。在7个月和10个月时，进行了发作间期SPECT检查；左侧半球的rCBF比右侧低45%。在随访期间，除了半球切除术后1个月观察到的轻度和短暂性下降外，右侧半球的全脑和局部CBF值显示正常水平和正常成熟度。SPECT为体内研究半球功能提供了一种额外的方法。系列SPECT成像可能对单侧脑畸形的术前和术后评估有用。
