Published online before print October 30, 2008, doi: 10.1161/STROKEAHA.108.515544
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(Stroke. 2008;39:3222.)
© 2008 American Heart Association, Inc.
Original Contributions |
Hemorrhage From Cavernous Malformations of the Brain
Definition and Reporting Standards
From the Division of Clinical Neurosciences (R.A.-S.S.), University of Edinburgh, UK; Strong Epilepsy Center (M.J.B.), University of Rochester Medical Center, NY; the Departments of Neurology and Pediatrics (L.M.), University of New Mexico, Albuquerque; and the Department of Neurological Surgery (I.A.A.), Northwestern University Feinberg School of Medicine, and Evanston Northwestern Healthcare, Ill.
Correspondence to Rustam Al-Shahi Salman, Bramwell Dott Building, Division of Clinical Neurosciences, Western General Hospital, Edinburgh. EH4 2XU, UK. E-mail Rustam.Al-Shahi{at}ed.ac.uk
Background and Purpose— Cavernous malformations of the brain (CMs) cause intracranial hemorrhage, but its reported frequency varies, partly attributable to study design. To improve the validity of future research, we aimed to develop a robust definition of CM hemorrhage.
Methods— We systematically reviewed the published literature (Ovid Medline and Embase to June 1, 2007) for definitions of CM hemorrhage used in studies of the untreated clinical course of 
20 participants with CM(s), to inform the development of a consensus statement on the clinical and imaging features of CM hemorrhage at a scientific workshop of the Angioma Alliance.
Results— A systematic review of 1426 publications about CMs in humans, revealed 15 studies meeting our inclusion criteria. Although 14 (93%) studies provided a definition of CM hemorrhage, data were less complete on the confirmatory type(s) of imaging (87%), whether CM hemorrhage should be clinically symptomatic (73%), and whether hemorrhage had to extend outside the CM or not (47%). We define a CM hemorrhage as requiring acute or subacute onset symptoms (any of: headache, epileptic seizure, impaired consciousness, or new/worsened focal neurological deficit referable to the anatomic location of the CM) accompanied by radiological, pathological, surgical, or rarely only cerebrospinal fluid evidence of recent extra- or intralesional hemorrhage. The definition includes neither an increase in CM diameter without other evidence of recent hemorrhage, nor the existence of a hemosiderin halo.
Conclusions— A consistent approach to clinical and brain imaging classification of CM hemorrhage will improve the external validity of future CM research.
Key Words: cerebral cavernous malformation • vascular malformations • stroke • hemorrhagic • genetics • KRIT1
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