OPTICUS-GLIOMAS IN 3 PATIENTS WITH VONREC KLINGHAUSENS-DISEASE

Background 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. Patients Three pat ients with gliomas of the optic nerve and chiasm with neurofibromatosi s 1 (NF1) were followed for 10-20 years. Clinical course and therapy a re discussed. Conclusion The correct diagnosis of exophthalmos, papill edema or optic atrophy in childhood is facilitated by family history a nd examination of an adult member of the family, who is likely to have already developed cafe-au-lait spots, neurofibromas of the skin, axil lary freckles and Lisch nodules as clinical signs of neurofibromatosis .