Hanefeld F, Kruse B, Holzbach U, Christen H J, Merboldt K D, Hänicke W, Frahm J
Zentrum Kinderheilkunde, Schwerpunkt Neuropädiatrie Georg-August-Universität, Göttingen, Germany.
Epilepsia. 1995 Dec;36(12):1215-24. doi: 10.1111/j.1528-1157.1995.tb01065.x.
Two children with hemimegalencephaly were examined by magnetic resonance imaging (MRI) and localized proton MR spectroscopy (MRS). In both cases, structural changes in the enlarged hemisphere included pachy- or polymicrogyria and gliosis of white matter. Associated metabolic disturbances included a dramatic reduction of glutamate and N-acetylaspartate (NAA) in white matter. Less severe or no alterations were noted in cortical gray matter, basal ganglia, and cerebellum. The older child (13 years) showed increased myoinositol in both gray and white matter as well as markedly increased choline-containing compounds in gray matter. Both children also had mildly decreased NAA levels in the white matter of the contralateral hemisphere. The spectroscopic findings indicate loss of vital neuroaxonal tissue and glial cell proliferation. Metabolic disturbances were more pronounced in the older child. The normal-appearing hemisphere was mildly affected in both cases.
对两名半侧巨脑回患儿进行了磁共振成像（MRI）检查和局部质子磁共振波谱分析（MRS）。在这两个病例中，增大半球的结构变化包括厚脑回或多小脑回以及白质胶质增生。相关的代谢紊乱包括白质中谷氨酸和N-乙酰天门冬氨酸（NAA）显著减少。皮质灰质、基底神经节和小脑的改变较轻或未发现改变。年龄较大的患儿（13岁）灰质和白质中的肌醇增加，灰质中含胆碱化合物明显增加。两名患儿对侧半球白质中的NAA水平也均轻度降低。波谱分析结果表明重要神经轴突组织丧失和胶质细胞增殖。年龄较大的患儿代谢紊乱更为明显。在这两个病例中，外观正常的半球均受到轻度影响。
