Cerebral vasculopathy in childhood neurofibromatosis type 2: cause for concern?

Developmental Medicine and Child Neurology
Karine LascellesVijeya Ganesan

Abstract

Unlike adult neurofibromatosis type 2 (NF2), which presents with symptoms related to bilateral vestibular schwannomas, children with NF2 most frequently present with ocular, dermatological, and neurological symptoms. Arteriopathy, a well-established feature in neurofibromatosis type 1, is not a widely recognized feature of NF2. Here we report three children with NF2 with cerebral arteriopathy and/or arterial ischaemic stroke. Bevacizumab, a vascular endothethial growth factor inhibitor, is an established treatment for rapidly growing vestibular schwannomas; however, it carries a risk of both ischaemic and haemorrhagic stroke. Thus, the role of screening and risk to benefit ratio of bevacizumab in NF2 merit further consideration. WHAT THIS PAPER ADDS: Children with neurofibromatosis type 2 (NF2) may be at increased risk of cerebral vasculopathy and arterial ischaemic stroke. Targeted magnetic resonance angiography should be performed in children with NF2 who are being considered for bevacizumab therapy.

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Citations

Jul 3, 2018·Developmental Medicine and Child Neurology·Fenella J KirkhamDawn E Saunders
Jan 23, 2019·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Claudia SantoroSilverio Perrotta
Aug 20, 2019·Current Opinion in Oncology·Dorothy HallidayD Gareth Evans
Apr 18, 2019·Clinical Genetics·Dorothy HallidayUNKNOWN English NF2 research group
Jul 9, 2020·Neurology. Genetics·John W HensonConnor McCormick
Aug 7, 2020·AJR. American Journal of Roentgenology·David DoigKshitij Mankad

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