F. Moro et N. Cavallaro, OPTICUS-GLIOMAS IN 3 PATIENTS WITH VONREC KLINGHAUSENS-DISEASE, Klinische Monatsblatter fur Augenheilkunde, 206(3), 1995, pp. 178-183
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
.