Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2017 Jun 2;12(6):e0178711.
doi: 10.1371/journal.pone.0178711. eCollection 2017.

Neurofibromatosis type 1 is not associated with subarachnoid haemorrhage

Arttu Kurtelius  1   2 , Roope A Kallionpää  3 , Jukka Huttunen  1   2 , Terhi J Huttunen  1   2 , Katariina Helin  1 , Timo Koivisto  1   2 , Juhana Frösen  1   2 , Mikael von Und Zu Fraunberg  1   2 , Sirkku Peltonen  4 , Juha Peltonen  3 , Juha E Jääskeläinen  1   2 , Antti E Lindgren  1   2
Affiliations

Neurofibromatosis type 1 is not associated with subarachnoid haemorrhage

Arttu Kurtelius et al. PLoS One. .

Abstract

Background: The prevalence of intracranial aneurysms (IAs) has been proposed to be elevated in the patients with neurofibromatosis type 1 (NF1). Our aims were to determine the prevalence of NF1 in a large Finnish population based cohort of IA patients and, on the other hand, the occurrences of subarachnoid haemorrhage and unruptured intracranial aneurysms in a nationwide population-based cohort of NF1 patients and its matched ten-fold control cohort.

Methods: The Kuopio IA Database (www.kuopioneurosurgery.fi) includes all ruptured and unruptured IA cases admitted to the Kuopio University Hospital (KUH) from its defined Eastern Finnish catchment population since 1980. In this registry-based study, we cross-linked the Kuopio IA database with the Finnish national registry covering all hospital diagnoses. The NF1 diagnoses of the 4543 patients with either saccular of fusiform IA were identified from 1969 to 2015 and verified from patient records. Our second approach was to analyze the occurrence of aneurysmal subarachnoid haemorrhage (aSAH) and unruptured IAs in a nationwide population-based database of 1410 NF1 patients and its ten-fold matched control cohort (n = 14030) using national registry of hospital diagnoses between 1987 and 2014.

Results: One NF1 patient was identified among the 4543 IA patients. Three verified IA cases (one unruptured IA and two aSAH cases) were identified in the cohort of 1410 NF1 patients, with similar occurrences in the control cohort.

Conclusions: We found no evidence in our population-based cohorts to support the conception that NF1 is associated with IAs. Our results indicate that the incidence of aSAH is not elevated in patients with NF1. Further studies are required to confirm that there is no association between NF1 and unruptured IAs.

PubMed Disclaimer

Conflict of interest statement

Competing Interests: The authors have declared that no competing interests exist.

References

    1. Upadhyaya M, Cooper D. Neurofibromatosis type 1: Molecular and cellular biology. Berlin Heidelberg: Springer-Verlag; 2012.
    1. Stumpf D, Alksne J, Annegers J. Neurofibromatosis. Conference statement. National institutes of health consensus development conference. Arch Neurol. 1988;45:575–578. - PubMed
    1. Jouhilahti E, Peltonen S, Heape AM, Peltonen J. The pathoetiology of neurofibromatosis 1. Am J Pathol. 2011;178:1932–1939. doi: 10.1016/j.ajpath.2010.12.056 - DOI - PMC - PubMed
    1. Rosser T, Vezina G, Packer R. Cerebrovascular abnormalities in a population of children with neurofibromatosis type 1. Neurology. 2005;64:553–555. doi: 10.1212/01.WNL.0000150544.00016.69 - DOI - PubMed
    1. Cairns AG, North KN. Cerebrovascular dysplasia in neurofibromatosis type 1. J Neurol Neurosurg Psychiatry. 2008;79:1165–1170. doi: 10.1136/jnnp.2007.136457 - DOI - PubMed

MeSH terms