2026 American Academy of Neurology Abstract Website

Objective:

Neurofibromatosis type 1 (NF1) is a common, heterogeneous neurocutaneous disorder characterized by a wide range of manifestations, from skin abnormalities to vascular complications. The incidence of cerebrovascular diseases (CVD) in NF1 can result from various pathologies, including ischemic stroke, aneurysm, and Moyamoya syndrome. Here, we present a case of medial longitudinal fasciculus (MLF) stroke in a patient with NF1 and discuss the multifactorial risk factors contributing to stroke in this condition.

Background:

A 37-year-old female with a history of NF1 presented with vertigo and numbness of the lower face. Neurological examination revealed left internuclear ophthalmoplegia (INO) without other focal deficits. Computed tomography angiography (CTA) of the brain and neck demonstrated patent carotid and vertebral arteries, with a hypoplastic right vertebral artery. Brain magnetic resonance imaging (MRI) revealed an acute infarction in the left posteromedial pons, involving the MLF. Mild vasogenic edema was also noted surrounding a superior cerebellar peduncle mass, first identified at age 27. She underwent craniectomy for biopsy at age 28, which revealed a grade II astrocytoma. The tumor has remained stable since her last radiation treatment nine years ago. It is worth noting that the cerebellar peduncle is a rare site for gliomas in NF1.

Design/Methods:

Results:

Firstly, hypoplasia of the right vertebral artery is one of the cerebrovascular abnormalities observed in NF1 and associated with ischemic stroke. Furthermore, radiation-induced vasculopathy can increase the risk of CVD within the radiation field, even after decades of radiation. Interestingly, the proximity of the astrocytoma to the infarct site, along with associated vasogenic edema, may represent a potential mechanism for vascular dysregulation and a prothrombotic state. Finally, the concurrent use of oral contraceptives in our patient poses an additional risk factor.

Conclusions:

Ischemic stroke in NF1 can result from a combination of vascular abnormalities, prior radiation, tumor-related edema, and traditional cerebrovascular risk factors.