Division of Neurology, Saitama Children's Medical Center, Saitama, Japan.
Epilepsia. 2010 Jul;51(7):1259-65. doi: 10.1111/j.1528-1167.2009.02495.x. Epub 2010 Feb 1.
To elucidate the abnormality of interictal regional cerebral blood flow (rCBF) of West syndrome at the onset.
Quantitative measurement of rCBF with an autoradiography method using N-isopropyl-((123)I) p-iodoamphetamine single photon emission computed tomography (SPECT) was performed on 14 infants with cryptogenic West syndrome. Regions of interest (ROIs) for rCBF were placed automatically using an automated ROI analysis software (three-dimensional stereotactic ROI template), and were grouped into 12 segments: callosomarginal, precentral, central, parietal, angular, temporal, posterior cerebral, pericallosal, lenticular nucleus, thalamus, hippocampus, and cerebellum. We compared rCBF between the patients and seven age-matched infants with cryptogenic focal epilepsy as a control group. The patients were divided into two groups according to the duration from onset to SPECT, to compare rCBF.
Quantitative analysis revealed cerebral hypoperfusion in cryptogenic West syndrome with normal SPECT images under visual inspection. In bilateral central, posterior cerebral, pericallosal, lenticular nucleus, and hippocampus, and in the left parietal, temporal, and cerebellum, and in the right angular and thalamus segments there were statistical differences (p < 0.05). Compared with the duration from onset to SPECT, there were no significant differences of rCBF in all segments.
Broad cerebral hypoperfusion with posterior predominance involving the hippocampus and lenticular nucleus implies that even cryptogenic West syndrome has a widespread cerebral dysfunction at least transiently, which would correspond to clinical manifestations of hypsarrhythmia and epileptic spasms. Hippocampal hypoperfusion suggests the dysfunction of hippocampal circuitry in the brain adrenal axis, and may contribute to subsequent cognitive impairment of cryptogenic West syndrome.
阐明婴儿痉挛症发作初期的发作间期局部脑血流（rCBF）异常。
采用 N-异丙基-（123I）碘代苯丙胺单光子发射计算机断层扫描（SPECT）的放射性自显影法对 14 例隐源性婴儿痉挛症患者进行 rCBF 定量测量。使用自动 ROI 分析软件（三维立体定向 ROI 模板）自动放置 rCBF 的感兴趣区（ROI），并将其分为 12 个节段：胼胝体缘、中央前回、中央、顶叶、角回、颞叶、大脑后、胼胝体下、纹状体、丘脑、海马和小脑。我们将患者的 rCBF 与 7 例年龄匹配的隐源性局灶性癫痫患者作为对照组进行比较。根据从发病到 SPECT 的时间将患者分为两组，以比较 rCBF。
定量分析显示，在视觉检查下正常 SPECT 图像的隐源性婴儿痉挛症存在脑灌注不足。双侧中央、大脑后、胼胝体下、纹状体和海马，以及左侧顶叶、颞叶和小脑，以及右侧角回和丘脑节段存在统计学差异（p<0.05）。与从发病到 SPECT 的时间相比，所有节段的 rCBF 均无显著差异。
广泛的以后部为主的脑灌注不足，包括海马和纹状体，提示即使是隐源性婴儿痉挛症至少在短暂时间内也存在广泛的脑功能障碍，这与高度失律和癫痫痉挛的临床表现相对应。海马灌注不足提示脑肾上腺轴中海马回路的功能障碍，可能导致隐源性婴儿痉挛症的随后认知障碍。
