This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by ter Berg, H. W. Articles by van Gijn, J. Search for Related Content PubMed PubMed Citation Articles by ter Berg, H. W. Articles by van Gijn, J.

Stroke, Vol 23, 1024-1030, Copyright © 1992 by American Heart Association

ARTICLES

Familial intracranial aneurysms. A review

HW ter Berg, DW Dippel, M Limburg, WI Schievink and J van Gijn
Department of Neurology, Twenteborg Hospital Almelo, The Netherlands.

BACKGROUND: A familial occurrence of intracranial aneurysms is defined by the presence of such aneurysms in two or more first to third-degree family members. Families with two affected members may represent accidental aggregation. Other families show a frequency compatible with an autosomal dominant mode of inheritance. A genetic basis is also suggested by the younger average age of familial cases with a ruptured intracranial aneurysm (42.3 years versus an age range of 50-54 years for nonfamilial cases), occurrence at the same site or a mirror site in sibling pairs, occurrence in identical twins, and the association of intracranial aneurysms with genetically transmitted disorders. SUMMARY OF REVIEW: No reliable data are available about the occurrence of familial intracranial aneurysms among all patients with ruptured aneurysms; a frequency of 6.7% has been reported from a retrospective study, but a large part of the "familial" occurrence can be explained by fortuitous aggregation. The pathogenesis of familial intracranial aneurysms is not fully explained; a (partial) deficiency of type III collagen has been reported in sporadic, but not in familial, cases. Clinical decision analysis shows how the risk of harboring an intracranial aneurysm and the age of the patient are the main determinants for elective screening; lifetime risk of rupture (and therefore age) and surgical risks are the determinants for neurosurgical treatment. CONCLUSIONS: Surgical treatment is recommended for patients aged less than 70 years with a moderate or low surgical risk, and screening (preferably by intra-arterial digital subtraction angiography) is recommended only for relatives aged 35-65 years. Magnetic resonance angiography may develop into a useful alternative for screening, but the risks of diagnostic procedures play only a minor role in the decision analysis.

This article has been cited by other articles:

				J van Beijnum, H B van der Worp, H M Schippers, O van Nieuwenhuizen, L J Kappelle, G J E Rinkel, J W B. van der Sprenkel, and C J M Klijn Familial occurrence of brain arteriovenous malformations: a systematic review J. Neurol. Neurosurg. Psychiatry, November 1, 2007; 78(11): 1213 - 1217. [Abstract] [Full Text] [PDF]

				R. D. Brown Jr. and D. G. Piepgras Screening for intracranial aneurysms after subarachnoid hemorrhage: Do our patients benefit? Neurology, February 10, 2004; 62(3): 354 - 356. [Full Text] [PDF]

				S. Graf, A. Schischma, K. E. Eberhardt, R. Istel, B. Stiasny, and B. D. Schulze Intracranial aneurysms and dolichoectasia in autosomal dominant polycystic kidney disease Nephrol. Dial. Transplant., May 1, 2002; 17(5): 819 - 823. [Abstract] [Full Text] [PDF]

				G.J. Hademenos, M.J. Alberts, I. Awad, M. Mayberg, T. Shephard, A. Jagoda, R.E. Latchaw, H.W. Todd, K. Viste, R. Starke, et al. Advances in the genetics of cerebrovascular disease and stroke Neurology, April 24, 2001; 56(8): 997 - 1008. [Abstract] [Full Text] [PDF]

				J. M. Wardlaw and P. M. White The detection and management of unruptured intracranial aneurysms Brain, February 1, 2000; 123(2): 205 - 221. [Abstract] [Full Text] [PDF]

				P. J Kirkpatrick and R S McConnell Screening for familial intracranial aneurysms BMJ, December 11, 1999; 319(7224): 1512 - 1513. [Full Text]

				K. Tsutsumi, K. Ueki, M. Usui, S. Kwak, and T. Kirino Risk of Subarachnoid Hemorrhage After Surgical Treatment of Unruptured Cerebral Aneurysms Stroke, June 1, 1999; 30(6): 1181 - 1184. [Abstract] [Full Text] [PDF]

				F. Crawley, A. Clifton, and M. M. Brown Should We Screen for Familial Intracranial Aneurysm? Stroke, February 1, 1999; 30(2): 312 - 316. [Abstract] [Full Text] [PDF]

				J. E C Bromberg, G. J E Rinkel, A. Algra, P. Greebe, C. M van Duyn, D. Hasan, M. Limburg, H. W M t. Berg, E. F M Wijdicks, and J. van Gijn Subarachnoid haemorrhage in first and second degree relatives of patients with subarachnoid haemorrhage BMJ, July 29, 1995; 311(7000): 288 - 289. [Full Text]

				J.E.C. Bromberg, G.J.E. Rinkel, A. Algra, M. Limburg, and J. van Gijn Outcome in Familial Subarachnoid Hemorrhage Stroke, June 1, 1995; 26(6): 961 - 963. [Abstract] [Full Text]