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Publisher Summary This chapter discusses the pathological aspects of cerebral dysplasia. Cerebral dysplasias, most commonly associated with seizure disorders, may be divided into two broad groups which include migration disorders and neuroectodermal dysplasias. Most individuals with a malformation of migration disorders group have seizure disorders, often difficult to control, and many are mentally retarded with or without more specific neurologic defects. The cerebral surface in micropolygyria condition may be composed of many small, irregular gyri separated by shallow sulci, or the gyral surface is unusually broad or even normal on external view. The relative increase in treatment of intractable seizure disorders by extirpative surgery has allowed pathologists to identify cortical malformations which are not otherwise recognizable by other diagnostic parameters. It is found that there are numerous subtypes of neuroectodermal dysplasias only a few of which are typically associated with a seizure disorder as a primary manifestation. The meningo-encephalo-angio-neurinomatosis (MEAN) disease which is composed of varying combinations of arachnoidal cells, blood vessels, and Schwann cells, and is most typically located over the surface of the brain, is also elaborated in the chapter. |