ABSTRACT

This chapter is divided into two sections: (1) Developmental malformations of the nervous system, and (2) Neurocutaneous disorders. Many of the neurocutaneous disorders could easily be considered in a discussion of tumors of the nervous system. However, several of the disorders show significant developmental abnormalities that justify their inclusion in this chapter. Furthermore, as discussed below, the nervous system emerges from the ectoderm of the primitive embryo from which the skin, as well as portions of the skull and face, also develops. As a consequence, germline or early somatic mutations of the ectoderm may produce defects in both the skin and nervous system resulting in neurocutaneous disorders.