Although case fatality after treatment has decreased over time, treatment of brain AVM remains associated with considerable risks and incomplete efficacy. Randomized controlled trials comparing different treatment modalities appear justified.

Spontaneous (non-traumatic) intracerebral haemorrhage (ICH) has a high case-fatality and leaves many survivors disabled. Clinical characteristics and outcome seem to vary according to the cause of ICH, but population-based comparisons are scarce. We studied two prospective, population-based cohorts to determine differences in outcome [case-fatality and modified Rankin Scale (mRS)] after incident ICH due to brain arteriovenous malformations (AVM) [Scottish Intracranial Vascular Malformation Study (SIVMS), n = 90] and spontaneous ICH [Oxford Vascular Study (OXVASC), n = 60]. Patients with AVM-ICH were younger, had lower pre-stroke and admission blood pressure (BP), higher admission Glasgow Coma Scale (GCS) and were more likely to have an ICH in a lobar location than patients with spontaneous ICH (sICH). Case fatality throughout 2-year follow-up was greater following sICH than AVM-ICH [34/56 (61%) versus 11/90 (12%) at 1 year, odds ratio (OR) 11 (95% Confidence Interval (CI) 5-25)], as was death or dependence (mRS >or= 3) [40/48 (83%) versus 26/65 (40%) at 1 year, OR 8 (3-19)]. Differences in outcome persisted following stratification by age and sensitivity analyses. In multivariable analyses of 1 year outcome, independent predictors of death were sICH (OR 21, 4-104) and increasing ICH volume (OR 1.03, 1.01-1.05), and independent predictors of death or dependence were sICH (OR 11, 2-62) and GCS on admission (OR 0.79, 0.67-0.93). Outcome after AVM-ICH is better than after sICH, independent of patient age and other known predictors of ICH outcome.

Background: Brain arteriovenous malformations (BAVMs) are thought to be sporadic developmental vascular lesions, but familial occurrence has been described. We compared the characteristics of patients with familial BAVMs with those of patients with sporadic BAVMs. Methods: We systematically reviewed the literature on patients with familial BAVMs. Three families that were found in our centre were added. Age, sex distribution and clinical presentation of the identified patients were compared with those in population based series of patients with sporadic BAVMs. Furthermore, we calculated the difference in mean age at diagnosis of parents and children to study possible anticipation. Results: We identified 53 patients in 25 families with BAVMs. Mean age at diagnosis of patients with familial BAVMs was 27 years (range 9 months to 58 years), which was younger than in the reference population (difference between means 8 years, 95% CI 3 to 13 years). Patients with familial BAVMs did not differ from the reference populations with respect to sex or mode of presentation. In families with BAVMs in successive generations, the age of the child at diagnosis was younger than the age of the parent (difference between means 22 years, 95% CI 13 to 30 years), which suggests clinical anticipation. Conclusions: Few patients with familial BAVMs have been described. These patients were diagnosed at a younger age than sporadic BAVMs whereas their mode of presentation was similar. Although there are indications of anticipation, it remains as yet unclear whether the described families represent accidental aggregation or indicate true familial occurrence of BAVMs.

Background and Purpose-It is not always clear whether, how, and when to undertake further radiological investigation of spontaneous (nontraumatic) intracerebral hemorrhage (ICH). Methods-We systematically reviewed Ovid MEDLINE and EMBASE databases for studies of the diagnostic utility of radiological investigations of the cause(s) of ICH. We sent a structured survey to neurologists, stroke specialists, neurosurgeons, and neuroradiologists in the United Kingdom, the Netherlands, and France to assess whether, how, and when they would investigate supratentorial ICH. Results-This systematic review detected 20 relevant studies (including 1933 patients), which either quantified the yield of a radiological investigation/imaging strategy (nϭ15) or compared 2 imaging techniques (nϭ5). Six hundred ninety-two (49%) physicians responded to the survey. Further investigation would have been undertaken by the following: 99% of respondents, for younger (38 to 43 years), normotensive adults with lobar or deep ICH; 76%, for older (age 72 to 83 years), normotensive adults with deep ICH; and 31%, for older adults with deep ICH and prestroke hypertension. Younger patient age was the strongest influence on the decision to further investigate ICH (odds ratioϭ16; 95% confidence interval, 13 to 20), followed by the absence of prestroke hypertension (odds ratioϭ5; 95% confidence interval, 4 to 6) and lobar ICH location (odds ratioϭ2; 95% confidence interval, 1 to 2). Conclusions-The