[Evolution of the growth of multiple intracranial tumors in a case of type 2 neurofibromatosis].

A sporadic case of neurofibromatosis type 2 in a 23-year old woman is described with clinical and neuroimaging (CT or MRI) 4 years follow-up. Together with bilateral acoustic tumours multiple intracranial tumours and solitary orbital meningioma were present, the latter being confirmed surgically and histopathologically. Ocular findings of congenital cataract but lack of other external stigmata of neurofibromatosis completed the clinical picture of the disease. Diagnostic criteria of NF2, difficulties in establishing the optimal management options are described and the importance of evaluating of family members is underlined. Neuroimaging pictures showed tumour expansion over 4 years leading to severe disability with total blindness, deafness, paresis and cerebellar syndrome.