Sankar R, Curran J G, Kevill J W, Rintahaka P J, Shewmon D A, Vinters H V
Department of Pediatrics, UCLA School of Medicine 90024, USA.
AJNR Am J Neuroradiol. 1995 Jun-Jul;16(6):1265-72.
To determine whether microscopic cortical lamination defects in patients with infantile spasms, not initially identifiable on MR, may be inferred from evolving changes in the adjacent white matter.
Three infants between 3 and 6 months of age presented with infantile spasms. Based on negative metabolic assessment and normal MR findings, they were classified as cryptogenic. Despite therapy the children deteriorated with seizure recurrence and the advent of lateralizing clinical and neurophysiologic findings. MR studies were repeated and positron emission tomography was done.
The second MR studies demonstrated abnormalities of myelination, corresponding to localized clinical and neurophysiologic findings. Positron emission tomography findings did not show a strong correlation; one was normal, one showed no abnormality in the major area of MR abnormality, and one showed significantly less abnormality than on MR. Two patients have undergone surgery, both with good response.
Subtle lamination defects may be identifiable on positron emission tomography but are usually not detectable on MR. White matter abnormality on MR images is usually attributable to primary disease. We suggest that in certain cases progressive white matter changes may be induced as a secondary phenomenon by overlying microscopic cortical lamination defects. Serial MR imaging may be beneficial in children with infantile spasms in whom signs of laterality evolve.
确定婴儿痉挛症患者最初在磁共振成像（MR）上无法识别的微观皮质分层缺陷是否可从相邻白质的演变变化中推断出来。
三名3至6个月大的婴儿出现婴儿痉挛症。基于代谢评估阴性和MR结果正常，他们被归类为隐源性。尽管进行了治疗，但患儿病情恶化，癫痫复发，并出现了临床和神经生理学定位体征。重复进行MR研究并进行正电子发射断层扫描（PET）。
第二次MR研究显示髓鞘形成异常，与局部临床和神经生理学发现相对应。PET结果没有显示出强烈的相关性；一个正常，一个在MR异常的主要区域未显示异常，一个显示的异常明显少于MR。两名患者接受了手术，均反应良好。
细微的分层缺陷在PET上可能可识别，但通常在MR上无法检测到。MR图像上的白质异常通常归因于原发性疾病。我们认为，在某些情况下，渐进性白质变化可能是由微观皮质分层缺陷作为继发现象引起的。对于婴儿痉挛症且出现定位体征演变的患儿，系列MR成像可能有益。
