Neurofibromatosis type 2. Case report.

A case of a 24-year-old woman with peripheral paresis of the facial nerve, balance disturbance, hearing loss and epileptic seizures for many years is presented. At time of admission to hospital cerebral magnetic resonance imaging (MRI) showed several large tumors situated supra- and infratentorially. Histological examination of the operated tumors revealed bilateral acoustic schwannomas in the cerebellopontine angles and mixed meningioma in the others. The patient was diagnosed as neurofibromatosis type 2 (NF2) according to clinical criteria for neurofibromatoses. Several months after the last operation, she exhibited weakness of all extremities. On spinal MRI an intramedullary tumor in the cervical region and additional focal lesions along central canal were found. Surgical therapy was not performed because of clinical improvement after dexamethasone treatment and location of lesion in cervical medulla. Our case confirms frequently occurring lack of neurocutaneous changes and late appearance of significant neurological symptoms in NF2.