The natural history of familial cerebral cavernomas: a retrospective MRI study of 40 patients

Our objective was to determine the natural history and prognostic factors o f familial forms of cerebral cavernous malformations (CCM). Cavernomas are one of the most common central nervous system vascular malformations. Famil ial CCM is increasingly diagnosed, but little is known about its natural hi story. In a national survey, we analysed clinical and MRI features of 173 p atients from 57 unrelated French families. Of these 40 had undergone at lea st two clinical and MRI examinations. Occurrence of haemorrhage, new lesion s, change in signal intensity and size of lesions have been studied by comp arison between first and last MRI studies. The CCM were classified accordin g to Zabramski et al. Mean follow-up was 3.2 years (range 0.5-6.5 years). W e followed 232 cavernomas (mean 5.9 per patient, range 1-17). Serial MRI de monstrated changes in 28 patients (70%). Bleeding occurred in 21 lesions (9 .1 %) in 14 patients (35 %). The haemorrhagic risk was 2.5 % per lesion-yea r, higher in type I and brain-stem CCM. We saw 23 new lesions appear in 11 patients (27.5 %), with an incidence of 0.2 lesions per patient year. Signa l change was observed in 11 patients (27.5 %), in 14 lesions (6 %), while 9 lesions (3.9 %) in 9 patients (22.5 %) changed significantly in size.