Predictors of cerebral cavernous malformation outcomes pinpointed

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By Eleanor McDermid, Senior medwireNews Reporter

A meta-analysis of individual patient data has identified risk factors for intracerebral haemorrhage (ICH) in patients with cerebral cavernous malformations (CCMs).

The highest 5-year risk, of 30.8%, was in patients with brainstem CCMs who had ICH or focal neurological deficits at the time of presentation, report Rustam Al-Shahi Salman (University of Edinburgh, UK) and study co-authors in The Lancet Neurology.

There were 495 such patients, as well as 327 who also presented with ICH or focal neurological deficits but had non-brainstem CCMs, and a lower 5-year ICH rate of 18.4%. Rates were lower still among patients without these symptoms at the time of diagnosis, at 8.0% for the 80 with brainstem CCMs and 3.8% for the 718 with non-brainstem CCMs.

The risk of ICH within 5 years was 4.4-fold higher among patients with brainstem than non-brainstem CCMs and 5.6-fold higher among those with ICH or new focal neurological deficits than among those without.

Of note, although patients with ICH or focal neurological deficits had a high risk of ICH during follow-up, this fell over time, from a risk of 6.2% during the first year after presentation to 2.0% in the fifth.

The researchers also assessed the effects of another three preselected risk factors – age, gender and having more than one CCM – but none of these predicted ICH.

In an accompanying commentary, Ale Algra and Gabriël Rinkel, from University Medical Center Utrecht in the Netherlands, say that the team’s “careful selection of predictors enhances applicability in clinical practice, in part because sophisticated laboratory tests are therefore not obligatory to allow prediction of risk.”

The patients in the study came from seven cohorts, covering North America, Europe and Asia. The researchers identified a further 1337 individuals (45% of all identified) in their literature search, but were unable to obtain the individual patient data.

Algra and Rinkel note that this makes external validation impossible, because all available data have been used to derive the risk factors, which is “a common problem with prognostic modelling for rare diseases.”

They say that the next challenge is to develop a decision analysis model that shows the effect of CCM treatment on the risk of ICH and death, to guide management of these patients.

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