Neurocutaneous Syndromes

This chapter will discuss neurocutaneous syndromes which is group of inherited and genetic disorders resulting in malproduction of several oncogenes that responsible for inhibition of tumor growth, these syndromes mainly involve the central nervous system and the skin but they may cause multiple symptoms in various organs of the body including the, the heart and the lungs, the eyes and many others. In this chapter we will discuss briefly the genetics and pathophysiology of the most famous and important of these syndromes including the Neurofibromatosis type 1, Neurofibromatosis type 2, tuberous sclerosis, Ataxia Telangiectasia, Sturge-Weber syndrome and some others. We will go through the clinical features regarding each system of the body and also the diagnostic criteria for each of these syndromes and also the etiology, we will go briefly through the management and the prognosis of these disorder as much of these has poor prognosis and the management is mostly palliative.