Vinters H V, De Rosa M J, Farrell M A
Department of Pathology (Neuropathology), UCLA Medical Center 90024-1732.
Epilepsia. 1993 Jul-Aug;34(4):772-9. doi: 10.1111/j.1528-1157.1993.tb00460.x.
Studies to date on the neuropathologic substrates of infantile spasms have largely utilized autopsy material of children who die after a long and complicated seizure history. This makes the interpretation of primary versus secondary changes in the cerebral tissue difficult if not impossible. We have recently had the opportunity to review the neuropathologic changes in cortical tissue resected from infants and children with a history of infantile spasms. The major identifiable abnormalities were destructive lesions, sometimes classifiable as cystic-gliotic encephalomalacia, and dysplastic changes of varying degree. The cortical dysplasias had some similarity to cerebral changes described in tuberous sclerosis, including the presence of bizarre gemistocytic "balloon" cells, and secondary cytoskeletal changes within neuronal cell bodies. Such material provides an opportunity to apply immunohistochemical and molecular techniques to epileptic tissue in an attempt to understand the morphologic substrates of infantile spasms and other types of generalized epilepsy.
迄今为止，关于婴儿痉挛症神经病理学基础的研究主要利用了那些在经历漫长而复杂的癫痫病史后死亡儿童的尸检材料。这使得即便并非不可能，也很难解释脑组织中的原发性变化与继发性变化。我们最近有机会回顾了从有婴儿痉挛症病史的婴幼儿切除的皮质组织中的神经病理学变化。主要可识别的异常是破坏性病变，有时可归类为囊性胶质软化性脑软化，以及不同程度的发育异常变化。皮质发育异常与结节性硬化症中描述的脑部变化有一些相似之处，包括存在奇异的肥胖性星形细胞样“气球”细胞，以及神经元细胞体内的继发性细胞骨架变化。这类材料为将免疫组织化学和分子技术应用于癫痫组织提供了机会，以试图了解婴儿痉挛症和其他类型全身性癫痫的形态学基础。
