Haginoya K, Kon K, Takayanagi M, Yoshihara Y, Kato R, Tanaka S, Yokoyama H, Munakata M, Nagai M, Maruoka S, Yamazaki T, Abe Y, Iinuma K
Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
Epilepsia. 1998;39 Suppl 5:26-9. doi: 10.1111/j.1528-1157.1998.tb05146.x.
We evaluated the ictal and interictal single photon emission computed tomography (SPECT) of 9 patients with West syndrome (WS). In this group, we noted two clear patterns of cortical hyperperfusion and subcortical hyperperfusion in the ictal SPECT. Both patterns were different from the previously documented ictal patterns for complex partial seizures (CPS) or secondarily generalized seizures. Our results suggest that the tonic spasms of WS do not always have a single neurophysiological basis; e.g., patients with hemihypsarrhythmia and focal hypsarrhythmia did not show ictal hyperperfusion of the lesion with hypsarrhythmia. These findings indicate that the origin of hypsarrhythmia as an EEG feature and the origin of tonic spasms may be different in such patients. In particular, hypsarrhythmia appears to originate from cortical lesions, whereas the subcortical structures may be primarily responsible for the tonic spasms. Our report is the first published study of ictal SPECT in patients with WS.
我们评估了9例韦斯特综合征（WS）患者的发作期和发作间期单光子发射计算机断层扫描（SPECT）。在该组患者中，我们在发作期SPECT中发现了两种明显的皮质血流灌注增加和皮质下血流灌注增加模式。这两种模式均与先前记录的复杂部分性发作（CPS）或继发性全身性发作的发作期模式不同。我们的结果表明，WS的强直痉挛并非总是具有单一的神经生理基础；例如，半侧高幅失律和局灶性高幅失律患者在发作期并未出现高幅失律病变部位的血流灌注增加。这些发现表明，在此类患者中，作为脑电图特征的高幅失律的起源与强直痉挛的起源可能不同。特别是，高幅失律似乎起源于皮质病变，而皮质下结构可能是强直痉挛的主要原因。我们的报告是关于WS患者发作期SPECT的首次发表研究。
