Abstract
Patients with neurofibromatosis demonstrate a very variable clinical picture with signs that are largely age dependent. Rarely a glioma is the first presenting sign of the disease. Three patients with gliomas of the optic nerve and chiasm with neurofibromatosis 1 (NF1) were followed for 10-20 years. Clinical course and therapy are discussed. The correct diagnosis of exophthalmos, papilledema or optic atrophy in childhood is facilitated by family history and examination of an adult member of the family, who is likely to have already developed café-au-lait spots, neurofibromas of the skin, axillary freckles and Lisch nodules as clinical signs of neurofibromatosis.
Published Version
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