Meningioangiomatosis without neurofibromatosis: a clinical analysis.

Abstract

Meningioangiomatosis is an uncommon clinical entity. This lesion has been reported at time of autopsy in patients with neurofibromatosis (NF) and in case reports of patients without NF Type 2 (NF2). The authors report a series of six patients with meningioangiomatosis who do not have NF2 and describe the clinical presentation, diagnosis of disease, and treatment. They also review the literature concerning this entity. Six patients with meningioangiomatosis were treated at the authors' institutions from 1994 to 2001. The mean age of the patients was 10.7 years (range 5-14 years). All of the children presented with a seizure disorder. Surgery was performed in all children, and a gross-total resection was accomplished. All patients exhibited clinical improvement. At last follow up (mean 6.3 years) all patients are free of seizures and are not taking anticonvulsant medications. No signs of recurrence have been noted on imaging studies. The authors advocate a gross-total resection of meningioangiomatosis for the treatment of seizure disorder in this population.