Animal models of focal cortical dysplasia and tuberous sclerosis complex: Recent progress toward clinical applications

被引:43
作者
Wong, Michael [1 ,2 ]
机构
[1] Washington Univ, Sch Med, Dept Neurol, St Louis, MO 63110 USA
[2] Washington Univ, Sch Med, Hope Ctr Neurol Disorders, St Louis, MO 63110 USA
基金
美国国家卫生研究院;
关键词
Mice; Rat; Epilepsy; Epileptogenesis; Malformation; EKER RAT MODEL; NEURONAL MIGRATION DISORDERS; MOUSE MODEL; CEREBRAL-LESIONS; MAMMALIAN TARGET; EPILEPSY; GLUTAMATE; EXPRESSION; RAPAMYCIN; MALFORMATIONS;
D O I
10.1111/j.1528-1167.2009.02295.x
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
P>Focal cortical dysplasia (FCD) and related malformations of cortical development (MCDs) represent an increasingly recognized cause of medically intractable epilepsy. However, the underlying mechanisms of epileptogenesis are poorly understood, and treatments for epilepsy due to various cortical malformations are often limited or ineffective. Animal models offer a number of advantages for investigating cellular and molecular mechanisms of epileptogenesis and developing novel, rational therapies for MCD-related epilepsy. This review highlights specific examples of how animal models have been useful in addressing several clinically relevant issues about epilepsy due to FCDs and related cortical malformations, including the pathologic and clinical features, etiologic factors, localization of the epileptogenic zone, neuronal and astrocytic contributions to epileptogenesis, and the development of antiepileptogenic therapies.
引用
收藏
页码:34 / 44
页数:11
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