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[30] [31] [32] The first brain region to be substantially affected is the striatum, followed by degeneration of the frontal and temporal cortices

Loss of heterozygosity of the NF1 gene in Schwann cells has been shown to be the necessary event for the development of discrete neurofibromas.8 Furthermore, neurofibromin-deficient Schwann cells secrete a substance which stimulates mast cell migration which, in turn, stimulates production of extracellular matrix and angiogenesis.9 Astrocytes lacking NF1 expression cannot form optic nerve gliomas on their own