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Familial intracranial aneurysms. A review
H W ter Berg1, D W Dippel, M Limburg
1Department of Neurology, Twenteborg Hospital Almelo, The Netherlands.
Stroke
|July 1, 1992
Summary
Familial intracranial aneurysms suggest a genetic link, with screening recommended for relatives aged 35-65. Surgical treatment is advised for patients under 70 with low to moderate risk.
Area of Science:
- Neurology
- Genetics
- Vascular Surgery
Background:
- Familial intracranial aneurysms involve two or more relatives, with some families exhibiting autosomal dominant inheritance patterns.
- Genetic factors are suggested by earlier onset in familial cases, consistent lesion sites in siblings, occurrence in identical twins, and links to genetic disorders.
- While a 6.7% frequency is reported, much familial occurrence may be coincidental aggregation.
Purpose of the Study:
- To review the definition, genetic basis, and clinical management of familial intracranial aneurysms.
- To analyze decision-making for screening and surgical treatment based on risk and age.
Main Methods:
- Review of existing literature on familial intracranial aneurysms.
- Clinical decision analysis incorporating risk of harboring an aneurysm, patient age, rupture risk, and surgical risks.
Main Results:
- The pathogenesis of familial intracranial aneurysms remains unclear; type III collagen deficiency is noted in sporadic, not familial, cases.
- Patient age and aneurysm risk are key for elective screening decisions.
- Rupture risk (age-dependent) and surgical risks guide neurosurgical treatment decisions.
Conclusions:
- Surgical intervention is recommended for patients under 70 with low to moderate surgical risk.
- Screening is advised for relatives aged 35-65, with intra-arterial digital subtraction angiography as a preferred method.
- Magnetic resonance angiography shows promise as a screening alternative, with diagnostic procedure risks having minimal impact on decisions.