A Case Of Multiple CNS Tumour

Phakomatosis is a group of disorder comprising of congenital hamartomatous malformations affecting the Central nervous system, skin and eye. Neurofibromatosis was described by Von Recklinghausen in 1882.Neurofibromatosis are of two types NF I and NF II, both are autosomal dominantly inherited condition. NF I is more common seen in 1 in 5000 whereas type NFII is found in 1 in 50,000. Neurofibromatosis consists of at least two dominantly inherited disordersneurofibromatosis 1 (NF1) and neurofibromatosis 2 (NF2). The criteria for their diagnosis have been clearly established.1 Both are known to have multi-system involvement. NF1 is associated with a variety of growth, puberty, and other endocrinologic disor-ders.2 NF2 is essentially a central nervous system disease. However, peripheral nerve tumors, dermatologic features, and ophthalmologic features may be present. Most of these patients present with hearing loss that may be accompanied or preceded by tinnitus. Ophthalmologic features are often asymptomatic.