Spinal MR findings in neurofibromatosis types 1 and 2.

PURPOSE To evaluate the frequency and nature of spinal pathology, the frequency of clinically silent lesions, and the potential benefit of screening spinal MR in neurofibromatosis patients. PATIENTS AND METHODS 28 neurofibromatosis type-1 (NF-1) patients and nine neurofibromatosis type-2 (NF-2) patients were studied with postcontrast spinal MR imaging. RESULTS NF-1: One patient had a biopsy-proven low-grade glioma; five patients, intradural, extramedullary masses (N = 23); one patient, extradural masses (N = 2) (neurofibromas); 16 patients had bony abnormalities; and three patients thecal sac abnormalities. NF-2: Five patients demonstrated intramedullary masses (five/eight ependymomas); nine patients, intradural, extramedullary masses (meningiomas, schwannomas); and four patients, bony abnormalities. Eight/10 NF-1 and four/nine NF-2 patients had asymptomatic masses. CONCLUSION Intradural disease is common, often asymptomatic, and often presents at a young age in NF-1 and NF-2 patients. Because of the propensity to develop significant asymptomatic as well as symptomatic intradural disease, screening of the entire spine with MR is recommended in both NF-1 and NF-2 patients.