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

Autor: Shazia K. Afridi, Vijeya Ganesan, Cheryl Hemingway, Karine Lascelles, Rosalie E. Ferner, Ata Siddiqui
Rok vydání: 2018
Předmět:
Zdroj: Developmental Medicine & Child Neurology. 60:1285-1288
ISSN: 0012-1622
DOI: 10.1111/dmcn.13920
Popis: 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.
Databáze: OpenAIRE