Untreated clinical course of cerebral cavernous malformations: a prospective, population-based cohort study

Rustam Al-Shahi Salman, Julie M Hall, Margaret A Horne, Fiona Moultrie, Colin B Josephson, Jo J Bhattacharya, Carl E Counsell, Gordon D Murray, Vakis Papanastassiou, Vaughn Ritchie, Richard C Roberts, Robin J Sellar, Charles P Warlow, for the Scottish Audit of Intracranial Vascular Malformations (SAIVMs) collaborators

Research output: Contribution to journalArticlepeer-review

224 Citations (Scopus)


BACKGROUND: Cerebral cavernous malformations (CCMs) are prone to bleeding but the risk of intracranial haemorrhage and focal neurological deficits, and the factors that might predict their occurrence, are unclear. We aimed to quantify these risks and investigate whether they are affected by sex and CCM location. METHODS: We undertook a population-based study using multiple overlapping sources of case ascertainment (including a Scotland-wide collaboration of neurologists, neurosurgeons, stroke physicians, radiologists, and pathologists, as well as searches of registers of hospital discharges and death certificates) to identify definite CCM diagnoses first made in Scottish residents between 1999 and 2003, which study neuroradiologists independently validated. We used multiple sources of prospective follow-up both to identify outcome events (which were assessed by use of brain imaging, by investigators masked to potential predictive factors) and to assess adults' dependence. The primary outcome was a composite of intracranial haemorrhage or focal neurological deficits (not including epileptic seizure) that were definitely or possibly related to CCM. FINDINGS: 139 adults had at least one definite CCM and 134 were alive at initial presentation. During 1177 person-years of follow-up (completeness 97%), for intracranial haemorrhage alone the 5-year risk of a first haemorrhage was lower than the risk of recurrent haemorrhage (2·4%, 95% CI 0·0-5·7 vs 29·5%, 4·1-55·0; p
Original languageEnglish
Pages (from-to)217-224
Number of pages8
JournalThe Lancet neurology
Issue number3
Early online date30 Jan 2012
Publication statusPublished - Mar 2012


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