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H W ter Berg

Publications and source records attributed to H W ter Berg.

7 recordsLinked to original sources

Familial intracranial aneurysms. A review.

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.

Decision Support Techniques

Screening for unruptured familial intracranial aneurysms: subarachnoid hemorrhage 2 years after angiography negative for aneurysms.

The screening of asymptomatic individuals in families with intracranial aneurysms has been advocated to detect unruptured aneurysms before a major hemorrhage occurs. We report a 39-year-old male member of a large Dutch family, with a documented history of intracranial aneurysms, who suffered a subarachnoid hemorrhage 2 years after cerebral digital subtraction angiography using intravenously administered contrast medium showed no abnormalities. Conventional arteriography demonstrated three intracranial aneurysms measuring 3 x 3 mm. Potential alternative screening procedures are discussed.

Adult

Decision analysis.

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Decision Making

Treatment of intact familial intracranial aneurysms: a decision-analytical approach.

Clinical decision analysis is applied to the treatment decisions for four patients with unruptured familial aneurysms. The surgical treatment was uneventful in all patients except one with mild mixed aphasia and facial weakness postoperatively; these deficits disappeared in less than 2 years. In the decision analysis, discounted Quality Adjusted Life Years are used as an outcome measure. Probability estimates are extracted from the literature when available. It is concluded that the decision to treat the aneurysm neurosurgically in three of the four patients was correct. In two of these three patients, the decision cannot be altered by plausible changes in estimated data. For the third patient, only the combination of a low probability of rupture, a high surgical mortality and morbidity, and high discount favors conservative treatment. In the fourth patient, a toss-up situation exists. More knowledge of the probability of rupture, the probability of the development of other aneurysms, and the results of operation on intact intracranial aneurysms would have made the analysis more accurate. Clinical research should address these issues.

Adult

Familial occurrence of intracranial aneurysms in childhood: a case report and review of the literature.

This report describes three individuals belonging to one family, who were affected with ruptured intracranial aneurysms (IAs) at a rather young age, 4, 15 and 23 years respectively. Familial IAs at this young age have not been described before. The evidence for a developmental, eventually inherited origin of familial IA in childhood is discussed with reference to the low occurrence of non-familial IA in childhood, IA-features in non-familial cases (localisation, multiplicity, size) and the association with (other) congenital malformations in the 0-5 years group.

Adolescent

Familial association of intracranial aneurysms and multiple congenital anomalies.

The familial occurrence of intracranial aneurysms and the possible relationship with connective tissue disease are discussed. We studied a large family in which seven members presented with aneurysms. Another family member presented with a subarachnoidal hemorrhage. Two other family members each presented with Marfan's syndrome and an unclassified multiple congenital anomalies syndrome, respectively. The multiplicity of the aneurysms in four members is in excess of that found in sporadic or familial cases with intracranial aneurysms. We suggest a common cause, eg, a connective tissue disorder for both the intracranial aneurysms, the Marfan's syndrome, and the unclassified syndrome.

Abnormalities, Multiple