Neuropathologic studies of cerebral cortical dysplasia.
- Author:
Eui Joo SOHN
1
;
Sei Jong KIM
;
Min Cheol LEE
;
Hyung Ihl KIM
Author Information
1. Department of Neurology, Chonnam University Medical School.
- Publication Type:Original Article
- MeSH:
Cell Differentiation;
Cytoplasm;
Glial Fibrillary Acidic Protein;
Humans;
Intermediate Filaments;
Malformations of Cortical Development*;
Mitochondria;
Neurons;
Seizures;
Silver;
Stem Cells;
Synapses;
Vimentin
- From:Journal of the Korean Neurological Association
1997;15(3):526-541
- CountryRepublic of Korea
- Language:Korean
-
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.