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  • Title: Intracranial abnormalities associated with facial plexiform neurofibromas in neurofibromatosis type 1.
    Author: Boltshauser E, Stocker H, Sailer H, Valavanis A.
    Journal: Neurofibromatosis; 1989; 2(5-6):274-7. PubMed ID: 2518508.
    Abstract:
    From 1975 to 1988 seventeen patients with neurofibromatosis type 1 and a disfiguring facial plexiform neurofibroma (FPN) were investigated. The FPN was left-sided in 13 patients. It was orbital/periorbital in 4, lower facial in 7 and involved the whole face in 6 subjects. Neuroimaging (n = 13) revealed a tumor (of optic pathways or basal ganglia) in 8, an ipsilateral middle cranial fossa arachnoid cyst in 2, multiple areas of high signal intensity (in T2-weighted magnetic resonance imaging) in 1, and normal findings in 2 patients. In NF-1 patients with FPN there seems to be a high incidence of intracranial tumors and possibly of arachnoid cysts. Our observation has to be confirmed in a larger patient series.
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