On the inheritance of intracranial aneurysms

Wouter I. Schievink, Daniel J Schaid, Harry M. Rogers, David G. Piepgras, Virginia V. Michels

Research output: Contribution to journalArticle

115 Citations (Scopus)

Abstract

Background and Purpose: The familial occurrence of intracranial aneurysms suggests the presence of a genetically determined underlying arteriopathy. The pattern of inheritance in these families usually is not known. Methods: A family with seven members with intracranial aneurysms is described and, from the literature before 1994, a total of 238 families with 560 affected members (56% female and 44% male) with intracranial aneurysms not associated with a known heritable disease are reviewed. A segregation analysis was performed on 73 of these families. Results: Two members were affected in the great majority of families (79%); five or more members were reported in only eight families (3%). The most common affected kinship was among siblings. Angiographic screening in 12 families detected an intracranial aneurysm in 29% of 51 asymptomatic relatives. Segregation analysis revealed several patterns of inheritance that were consistent with the compiled pedigrees, but no single mendelian model was the overall best fitting, suggesting that genetic heterogeneity may be important. Twenty-two percent of siblings of male probands had an intracranial aneurysm compared with 9% of siblings of female probands (P=.003). Conclusions: Genetic heterogeneity may be important in the genetics of intracranial aneurysms. In families with intracranial aneurysms, siblings of an affected male proband may be at a higher risk of developing an aneurysm than siblings of an affected female proband. Screening for intracranial aneurysms in asymptomatic relatives should be considered in families with two or more affected members. In most families, the nature of the underlying arteriopathy remains obscure.

Original languageEnglish (US)
Pages (from-to)2028-2037
Number of pages10
JournalStroke
Volume25
Issue number10
StatePublished - Oct 1994

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Intracranial Aneurysm
Inheritance Patterns
Genetic Heterogeneity
Pedigree
Aneurysm

Keywords

  • cerebral aneurysm
  • genetics
  • subarachnoid hemorrhage

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Neuroscience(all)

Cite this

Schievink, W. I., Schaid, D. J., Rogers, H. M., Piepgras, D. G., & Michels, V. V. (1994). On the inheritance of intracranial aneurysms. Stroke, 25(10), 2028-2037.

On the inheritance of intracranial aneurysms. / Schievink, Wouter I.; Schaid, Daniel J; Rogers, Harry M.; Piepgras, David G.; Michels, Virginia V.

In: Stroke, Vol. 25, No. 10, 10.1994, p. 2028-2037.

Research output: Contribution to journalArticle

Schievink, WI, Schaid, DJ, Rogers, HM, Piepgras, DG & Michels, VV 1994, 'On the inheritance of intracranial aneurysms', Stroke, vol. 25, no. 10, pp. 2028-2037.
Schievink WI, Schaid DJ, Rogers HM, Piepgras DG, Michels VV. On the inheritance of intracranial aneurysms. Stroke. 1994 Oct;25(10):2028-2037.
Schievink, Wouter I. ; Schaid, Daniel J ; Rogers, Harry M. ; Piepgras, David G. ; Michels, Virginia V. / On the inheritance of intracranial aneurysms. In: Stroke. 1994 ; Vol. 25, No. 10. pp. 2028-2037.
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