Neuropathologic studies of cerebral cortical dysplasia

J Korean Neurol Assoc > Volume 15(3); 1997 > Article

Journal of the Korean Neurological Association 1997;15(3): 526-541.

대뇌피질 이형성증의 신경병리학적 소견

손의주, 김세종, 이민철, 김형일

전남대학교 의대 신경과. 병리학과, 전북대학교 의대 신경외과

Neuropathologic studies of cerebral cortical dysplasia

Eui-Joo Sohn, Sei-Jong Kim, Min-Cheol Lee, Hyung-Ihl Kim

Department of Neurology and Pathology, Chonnam University Medical School, Department of Neurosurgery, Chonbuk University School of Medicine

Abstract

Cortical dysplasia(CD) represents a spectrum of neuropathologic changes reflecting a derangement of the normal process of neocortical development. We have presented 32 patients who underwent cortical recectiom for intractable seizures and demonstrated the neuropathologic features, which could be explained by a disturbance in the process of neural development in the farm. It could be characterized by light microscopic features: cortical laminar disorganization, neurons in the molecular layer, subpial re=ants of granule calls, remnants of marginal glioneuronal heterotopia, neuronal heterotopia in the white matter, polymicrogyria, neuronal cytomegaly and balloon cell change. Even though cortical dyslamimtion was the consistent finding of all the cases, the neuronal cytomegaly and balloon cell change were diagnostic hallmarks in the study. The cytomegatic neurons were strongly reactive to silver impregration and to immunohistochemical marrkers of neurons, such as neurofilament protein (NF, 68 and 200 kDa) and neuron-specific enolase(NSE). They showed hypertrophic endoplmmic reticul= and increased number of mitochondria in their cytoplasm and incomplete synapses in electron microscopic study. The balloon cells were positively stained by glial fibrillary acidic protein, NSE and vimentin and were filled with intermediate filaments in their cytoplasm. These results indicated that both cytomegalic neurons and balloon cells are produced by faulty cell differentiation involving neuroblast in the former, and both neuronal and glial stem cell lines in the latter.