[Multiple intracranial and intraspinal meningiomas in the neurocristopathy (phacomatosis) type of neurofibromatosis].

Presentation of a case of a "central type" neurofibromatosis in a 56-year old woman, clinically diagnosed erroneously as multiple sclerosis with a 20 years long course. Disturbances of hearing, walking, sight, sensitivity, incontinentia, intracranial hypertension and headache represented the main symptoms. More than 120 intracranial and tens of intraspinal meningiomas represented the leading postmortem finding. In a lesser frequency spinal plexiform neurofibromas and schwannomas were also found. The death was attributed to aspiration purulent bronchopneumonia. Various types of meningioma were seen microscopically, including secretory type and a type with amyloid. Immunostaining was positive with S-100 protein and EMA. Negative expression was found with vimentin, CEA, smooth muscle actin, estrogen and progesterone receptors, amyloid A and cytokeratins. With regard to the presence or absence of key morphological features the presented case was placed according to Sobol et al. (29) into the seventh category of neurofibromatosis (NF7).