title: Prevalence of brain arteriovenous malformations in first-degree relatives of patients with a brain arteriovenous malformation.
abstract: OBJECTIVE
  It is uncertain whether familial occurrence of brain arteriovenous malformations (BAVMs) represents coincidental aggregation or a shared familial risk factor. We aimed to compare the prevalence of BAVMs in first-degree relatives (FDRs) of patients with BAVM and the prevalence in the general population.
  
  
  METHODS
  We sent a postal questionnaire to 682 patients diagnosed with a BAVM in 1 of 4 university hospitals to retrieve information about the occurrence of BAVMs among their FDRs. We calculated a prevalence ratio using the BAVM prevalence among FDRs and the prevalence from a Scottish population-based study (93 per 628 788 adults). A prevalence ratio of ≥9 with a lower limit of the 95% confidence interval of 3 was considered indicative of a shared familial risk factor.
  
  
  RESULTS
  Informed consent was given by 460 (67%) patients, who had 2992 FDRs. We identified 3 patients with a FDR with a BAVM, yielding a prevalence ratio of 6.8 (95% CI, 2.2-21).
  
  
  CONCLUSIONS
  The prevalence of BAVMs in FDRs of patients with a BAVM was increased but did not meet our prespecified criterion for a shared familial risk factor. In combination with the low absolute risk of a BAVM in FDRs, our results do not support screening of FDRs for BAVMs.
authors.affiliation: From the Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus (J.v.B., H.B.v.d.W., A.A., J.W.B.v.d.S., L.J.K., G.J.E.R., C.J.M.K.), and the Julius Centre for Health Science and Primary Care (A.A.), University Medical Center Utrecht, Utrecht, The Netherlands; Departments of Neurosurgery (W.P.V.) and Radiology (R.v.d.B.), Neurosurgical Center Amsterdam, VU University Medical Center and Amsterdam Medical Center, Amsterdam, The Netherlands; and Departments of Neurosurgery (J.v.B.) and Radiology (P.A.B.), Leiden University Medical Center, Leiden, The Netherlands. J.vanBeijnum@gmail.com.
authors.forename: Janneke
authors.initials: J
authors.lastname: van Beijnum
authors.affiliation: From the Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus (J.v.B., H.B.v.d.W., A.A., J.W.B.v.d.S., L.J.K., G.J.E.R., C.J.M.K.), and the Julius Centre for Health Science and Primary Care (A.A.), University Medical Center Utrecht, Utrecht, The Netherlands; Departments of Neurosurgery (W.P.V.) and Radiology (R.v.d.B.), Neurosurgical Center Amsterdam, VU University Medical Center and Amsterdam Medical Center, Amsterdam, The Netherlands; and Departments of Neurosurgery (J.v.B.) and Radiology (P.A.B.), Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: H Bart
authors.initials: HB
authors.lastname: van der Worp
authors.affiliation: From the Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus (J.v.B., H.B.v.d.W., A.A., J.W.B.v.d.S., L.J.K., G.J.E.R., C.J.M.K.), and the Julius Centre for Health Science and Primary Care (A.A.), University Medical Center Utrecht, Utrecht, The Netherlands; Departments of Neurosurgery (W.P.V.) and Radiology (R.v.d.B.), Neurosurgical Center Amsterdam, VU University Medical Center and Amsterdam Medical Center, Amsterdam, The Netherlands; and Departments of Neurosurgery (J.v.B.) and Radiology (P.A.B.), Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: Ale
authors.initials: A
authors.lastname: Algra
authors.affiliation: From the Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus (J.v.B., H.B.v.d.W., A.A., J.W.B.v.d.S., L.J.K., G.J.E.R., C.J.M.K.), and the Julius Centre for Health Science and Primary Care (A.A.), University Medical Center Utrecht, Utrecht, The Netherlands; Departments of Neurosurgery (W.P.V.) and Radiology (R.v.d.B.), Neurosurgical Center Amsterdam, VU University Medical Center and Amsterdam Medical Center, Amsterdam, The Netherlands; and Departments of Neurosurgery (J.v.B.) and Radiology (P.A.B.), Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: W Peter
authors.initials: WP
authors.lastname: Vandertop
authors.affiliation: From the Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus (J.v.B., H.B.v.d.W., A.A., J.W.B.v.d.S., L.J.K., G.J.E.R., C.J.M.K.), and the Julius Centre for Health Science and Primary Care (A.A.), University Medical Center Utrecht, Utrecht, The Netherlands; Departments of Neurosurgery (W.P.V.) and Radiology (R.v.d.B.), Neurosurgical Center Amsterdam, VU University Medical Center and Amsterdam Medical Center, Amsterdam, The Netherlands; and Departments of Neurosurgery (J.v.B.) and Radiology (P.A.B.), Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: René
authors.initials: R
authors.lastname: van den Berg
authors.affiliation: From the Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus (J.v.B., H.B.v.d.W., A.A., J.W.B.v.d.S., L.J.K., G.J.E.R., C.J.M.K.), and the Julius Centre for Health Science and Primary Care (A.A.), University Medical Center Utrecht, Utrecht, The Netherlands; Departments of Neurosurgery (W.P.V.) and Radiology (R.v.d.B.), Neurosurgical Center Amsterdam, VU University Medical Center and Amsterdam Medical Center, Amsterdam, The Netherlands; and Departments of Neurosurgery (J.v.B.) and Radiology (P.A.B.), Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: Patrick A
authors.initials: PA
authors.lastname: Brouwer
authors.affiliation: From the Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus (J.v.B., H.B.v.d.W., A.A., J.W.B.v.d.S., L.J.K., G.J.E.R., C.J.M.K.), and the Julius Centre for Health Science and Primary Care (A.A.), University Medical Center Utrecht, Utrecht, The Netherlands; Departments of Neurosurgery (W.P.V.) and Radiology (R.v.d.B.), Neurosurgical Center Amsterdam, VU University Medical Center and Amsterdam Medical Center, Amsterdam, The Netherlands; and Departments of Neurosurgery (J.v.B.) and Radiology (P.A.B.), Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: Jan Willem Berkelbach
authors.initials: JW
authors.lastname: van der Sprenkel
authors.affiliation: From the Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus (J.v.B., H.B.v.d.W., A.A., J.W.B.v.d.S., L.J.K., G.J.E.R., C.J.M.K.), and the Julius Centre for Health Science and Primary Care (A.A.), University Medical Center Utrecht, Utrecht, The Netherlands; Departments of Neurosurgery (W.P.V.) and Radiology (R.v.d.B.), Neurosurgical Center Amsterdam, VU University Medical Center and Amsterdam Medical Center, Amsterdam, The Netherlands; and Departments of Neurosurgery (J.v.B.) and Radiology (P.A.B.), Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: L Jaap
authors.initials: LJ
authors.lastname: Kappelle
authors.affiliation: From the Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus (J.v.B., H.B.v.d.W., A.A., J.W.B.v.d.S., L.J.K., G.J.E.R., C.J.M.K.), and the Julius Centre for Health Science and Primary Care (A.A.), University Medical Center Utrecht, Utrecht, The Netherlands; Departments of Neurosurgery (W.P.V.) and Radiology (R.v.d.B.), Neurosurgical Center Amsterdam, VU University Medical Center and Amsterdam Medical Center, Amsterdam, The Netherlands; and Departments of Neurosurgery (J.v.B.) and Radiology (P.A.B.), Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: Gabriël J E
authors.initials: GJ
authors.lastname: Rinkel
authors.affiliation: From the Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus (J.v.B., H.B.v.d.W., A.A., J.W.B.v.d.S., L.J.K., G.J.E.R., C.J.M.K.), and the Julius Centre for Health Science and Primary Care (A.A.), University Medical Center Utrecht, Utrecht, The Netherlands; Departments of Neurosurgery (W.P.V.) and Radiology (R.v.d.B.), Neurosurgical Center Amsterdam, VU University Medical Center and Amsterdam Medical Center, Amsterdam, The Netherlands; and Departments of Neurosurgery (J.v.B.) and Radiology (P.A.B.), Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: Catharina J M
authors.initials: CJ
authors.lastname: Klijn
mesh_terms: D000293:Adolescent; D000328:Adult; D001164:Arteriovenous Fistula / Q000175:diagnosis* / Q000453:epidemiology*; D005190:Family; D005260:Female; D006801:Humans; D002538:Intracranial Arteriovenous Malformations / Q000175:diagnosis* / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012307:Risk Factors; D011795:Surveys and Questionnaires; D055815:Young Adult
keywords: family; intracranial arteriovenous malformations