Serial MRIs provide novel insight into natural history of optic pathway gliomas in patients with neurofibromatosis 1

• DOI: 10.1186/s13023-018-0811-9
• Publication Date: 2018-04-23T12:02:35Z
• Authors: Laura Sellmer; Said Farschtschi; Marco Marangoni; Manraj K. S. Heran; Patricia Birch; Ralph Wenzel; Victor-Felix Mautner; Jan M. Friedman
• Keywords: Optic chiasm

Optic pathway gliomas (OPGs) are present in 20% of children with neurofibromatosis 1 (NF1) but less frequently observed adults. Our goal was to determine the natural history OPGs and adults NF1.We analyzed features other intracranial lesions on 1775 head MRI scans 562 unselected NF1 collected between 2003 2015. 52 (9.3%) patients this study had an OPG diagnosed their MRI. The median age at first scan 12.7 years. Of patients, intraorbital optic nerves were affected 29 (56%), prechiasmatic 32 (62%), chiasm 17 (33%) radiations 19 (37%). two or more areas affected. One patient a newly-appearing OPG, showed progression. rate progression over 5 years 2.4% (95% CI: 0.4% 16%). Four partial regression OPGs, we no case complete during study. 8.9% confidence intervals: 2.8% 26%). We found presence UBOs individual be highly associated (p = 0.0061).OPGs common older than previously thought. occurrences unidentified bright objects (UBOs) asymptomatic each other. This suggests possibility that remain may differ pathogenically from those become symptomatic.