Neurovascular Issues in Neurofibromatosis Type I: Focus on Intracranial Stenosis

Background/Objectives: Neurofibromatosis type 1 (NF1) is a genetic disorder characterized by various clinical manifestations, including significant neurovascular complications. This review aims to synthesize current knowledge regarding intracranial stenoses and associated vascular abnormalities in patients with NF1, emphasizing the differences between pediatric and adult populations. Methods: A narrative review was conducted, analyzing the existing literature on the epidemiology, clinical manifestations, and management of neurovascular issues related to NF1. Data were collected from a range of studies, including retrospective analyses and case series, focusing on the incidence and outcomes of intracranial vascular abnormalities. Results: The study found that intracranial vasculopathy affects between 0.4% and 6.4% of NF1 patients, with children experiencing higher rates of stenotic lesions. However, vascular issues in adults are less understood, with 3.5% of adult patients presenting vascular abnormalities. The review highlights a significant underdiagnosis of these conditions due to the lack of routine use of magnetic resonance angiography (MRA) in standard evaluations. The management of NF1-related vascular conditions, particularly in adults, remains poorly defined, particularly regarding the efficacy of antithrombotic therapies. Conclusions: The management of neurovascular complications in NF1 requires urgent attention, with a need for standardized screening protocols and further research to elucidate the natural history and optimal treatment strategies for these patients. Enhanced diagnostic practices, including routine neuroimaging, are essential to improve outcomes and reduce the risk of significant vascular events.