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带状灰质异位症的功能性神经放射学检查

Functional neuroradiologic investigations in band heterotopia.

作者信息

Iannetti P, Spalice A, Raucci U, Perla F M

机构信息

Division of Pediatric Neurology, Pediatrics Department, "La Sapienza" University, Roma, Italy.

出版信息

Pediatr Neurol. 2001 Feb;24(2):159-63. doi: 10.1016/s0887-8994(00)00247-2.

Abstract

Band heterotopias are an example of genetic generalized neuronal migration disorders that may be present in patients with mild epilepsy and normal or slightly impaired intellect, as well as in patients with intractable epilepsy and mental retardation. The case of a 17-year-old left-handed female patient with epilepsy and normal cognitive development is reported in whom single-photon emission computed tomography (SPECT), proton magnetic resonance spectroscopy, and functional magnetic resonance imaging (fMRI) were performed. MRI revealed the presence of bilateral asymmetric band heterotopia. SPECT revealed a left frontoparietal and occipital hypoperfusion, demonstrating a good correlation with the electroencephalogram abnormalities. Because of the appearance of new types of seizures, the patient underwent a second MRI investigation together with a proton magnetic resonance spectroscopy (MRS) study. MRI confirmed bilateral band heterotopia characterized by greater thickness in the left hemisphere at the frontal and occipital level. MRI and SPECT findings were in agreement with left occipital electroencephalogram abnormalities and with occipital seizure type. Qualitative results of proton MRS revealed normal spectra profiles in the examined left frontal and occipital heterotopic area and in the normal overlying cortex. Later, fMRI was performed. The finger-tapping test of the right hand yielded the activation of both normal left sensory-motor cortex and the facing band heterotopia. In the right hemisphere, only the activation of the sensory-motor neocortex was observed; no involvement of the right misplaced brain tissue was present. This functional behavior could be considered the consequence of poor neuronal representation. On the contrary, the involvement of both band heterotopia and normal cortex observed in the left hemisphere could be the result of many synaptic interconnections. Functional investigations may have an important role in defining the activity of band heterotopia per se and in relation to the overlying neocortex.

摘要

带状异位是遗传性广泛性神经元迁移障碍的一个例子,可能存在于轻度癫痫且智力正常或稍有受损的患者中,也见于难治性癫痫和智力发育迟缓的患者。本文报道了一名17岁左利手女性癫痫患者,其认知发育正常,对该患者进行了单光子发射计算机断层扫描(SPECT)、质子磁共振波谱分析和功能磁共振成像(fMRI)检查。MRI显示存在双侧不对称带状异位。SPECT显示左额顶叶和枕叶灌注不足,与脑电图异常表现出良好的相关性。由于出现了新型癫痫发作,该患者接受了第二次MRI检查及质子磁共振波谱(MRS)研究。MRI证实双侧带状异位,其特征为额叶和枕叶水平的左半球厚度更大。MRI和SPECT结果与左枕叶脑电图异常及枕叶癫痫类型相符。质子MRS的定性结果显示,在所检查的左额叶和枕叶异位区域以及正常覆盖皮质中,光谱轮廓正常。随后进行了fMRI检查。右手的手指敲击测试激活了正常的左感觉运动皮质和相对应的带状异位。在右半球,仅观察到感觉运动新皮质的激活;未发现右位错脑组织受累。这种功能行为可被认为是神经元代表不足的结果。相反,在左半球观察到的带状异位和正常皮质均受累可能是许多突触连接的结果。功能研究可能在确定带状异位本身的活动以及与覆盖新皮质的关系方面发挥重要作用。

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