Neuronal Cytoskeletal Abnormalities and Neurotrophin Receptor Immunoreactivity in Severe Cerebral Cortical Dysplasia

J Korean Neurol Assoc > Volume 18(2); 2000 > Article

Journal of the Korean Neurological Association 2000;18(2): 199-210.

중증 대뇌피질이형성증에서 신경세포골격이상 및 Neurotrophin 수용체 발현

김주용, 노재규*·정천기**

강원대학교 의과대학 신경과,서울대학교 의과대학 신경과*,신경외과**

Neuronal Cytoskeletal Abnormalities and Neurotrophin Receptor Immunoreactivity in Severe Cerebral Cortical Dysplasia

Joo-Yong Kim, M.D., Jae-Kyu Roh, M.D.*, Chun-Kee Chung, M.D.**

Department of Neurology, College of Medicine, Kangwon National University Department of Neurology* and Neurosurgery**, College of Medicine, Seoul National University

Abstract

Background : Cerebral cortical dysplasia (CD) is one of the important causes of intractable epilepsies and character-ized histologically by disorganized cortical lamination and cytomegalic dysplastic neurons. Although various cytoskeletal abnormalities have been found in dysplastic neurons of CD, the pathogenetic role of dysplastic neurons has rarely been investigated. M e t h o d s : In this study, immunohistochemical analysis was performed using antibodies against non-phosphorylated high- or medium- molecular weight neurofilament protein and microtubule-associated pro-tein 2 (MAP-2) in surgical specimens of CD. In order to know the possible relationship of dysplastic neurons with cytoskeletal abnormalities and various neurotrophin receptors, NGFR p75, trkA, trkB, and trkC immunoreactivities were also analyzed. Results : Dysplastic neurons showed strong immunoreactivities for non-phosphorylated high- or medium- molecular weight neurofilament protein and MAP-2, which might reflect abnormal outgrowth and altered plasticity of the dysplastic neurons. TrkB and trkC were strongly expressed in dysplastic neurons and NGFR p75 was also strongly expressed in some dysplastic neurons. C o n c l u s I o n s : Since it has been known that brain-derived neu-rotrophic factor (BDNF) and neurotrophin-3 (NT-3) have effects on the differentiation of neuronal precursor cells from the cortex and on dendritic and axonal arborization, increased expression of trkB and trkC may play a role in cytoskele-tal abnormalities and altered synaptic transmission in dysplastic neurons. J Korean Neurol Assoc 18(2):199~210, 2000 Key Words : Cortical dysplasia, Cytomegalic neuron, Cytoskeletal protein, Neurotrophin, Receptor