Karagöl U, Deda G, Uysal S, Kabakus N, Ibis E, Gençoglu A, Aras G
Department of Pediatrics, Ankara University Faculty of Medicine, Turkey.
Pediatr Int. 2001 Feb;43(1):66-70. doi: 10.1046/j.1442-200x.2001.01330.x.
Infantile spasm (IS) is an age-dependent epileptic encephalopathy of variable etiology. Although IS is well studied, its pathogenesis is unclear. Infantile spasm is usually considered a generalized epilepsy, but recent studies point to focal cerebral blood flow (CBF) abnormalities.
In six symptomatic IS patients, single photon emission computed tomography (SPECT) with [99Tc]-HMPAO, electroencephalography (EEG), magnetic resonance imaging (MRI)/computed tomography (CT) and their correlation were evaluated.
Single photon emission computed tomography showed unifocal (regional) cerebral hypoperfusion in two infants, multifocal (diffuse) cerebral hypoperfusion in three infants and normal perfusion in the other infant. Electroencephalograms obtained in near-time of the SPECT studies showed diffuse abnormalities in five infants with hypoperfusion on SPECT. Cranial MRI/CT showed diffuse and/or localized cerebral lesions in all infants, all of whom had corresponding areas of hypoperfusion on SPECT. In one patient whose spasms were stopped with anticonvulsants, SPECT was normal, in two patients SPECT showed unifocal lesions, while in another three patients whose spasms were decreased but not stopped, SPECT showed multifocal hypoperfusion.
This pilot study may indicate that there are CBF anomalies in symptomatic IS. The degree of CBF may be a predictor of prognosis and multifocal hypoperfusion may be a poor prognostic criteria in IS.
婴儿痉挛症（IS）是一种病因多样的年龄依赖性癫痫性脑病。尽管对婴儿痉挛症已有充分研究，但其发病机制仍不清楚。婴儿痉挛症通常被认为是一种全身性癫痫，但最近的研究指出存在局灶性脑血流量（CBF）异常。
对6例症状性婴儿痉挛症患者进行了[99Tc]-六甲基丙烯胺肟单光子发射计算机断层扫描（SPECT）、脑电图（EEG）、磁共振成像（MRI）/计算机断层扫描（CT）检查，并评估了它们之间的相关性。
SPECT显示，2例婴儿为单灶性（局部）脑灌注不足，3例为多灶性（弥漫性）脑灌注不足，另1例灌注正常。在SPECT检查临近时间获得的脑电图显示，5例SPECT灌注不足的婴儿存在弥漫性异常。头颅MRI/CT显示所有婴儿均有弥漫性和/或局限性脑病变，且所有婴儿在SPECT上均有相应的灌注不足区域。1例使用抗惊厥药物后痉挛停止的患者SPECT正常，2例患者SPECT显示单灶性病变，另外3例痉挛减轻但未停止的患者SPECT显示多灶性灌注不足。
这项初步研究可能表明，症状性婴儿痉挛症存在脑血流量异常。脑血流量程度可能是预后的一个预测指标，多灶性灌注不足可能是婴儿痉挛症预后不良的一个标准。
