J Neurosurg 144:1344–1352, 2026
This prospective cohort study quantifies long-term functional outcomes in 332 untreated patients with cerebral cavernous malformation (CCM), using modified Rankin Scale (mRS) scores to evaluate disability over a mean 6.6-year follow-up. It reports incidence of symptomatic hemorrhage (SH), temporal patterns of recovery after a first SH, and external validation of published predictive nomograms.
Key findings identify brainstem location, a history of self-reported psychiatric disorder, and two or more SHs as independent predictors of long-term disability; most patients improved within one year after a first hemorrhage, and ten-year disability risk for nonbrainstem CCM was under 8%.
Objective Elucidate long-term morbidity and disability risk in adults with untreated cerebral cavernous malformation (CCM).
Methods Prospectively followed registry cohort; functional outcome tracked using mRS, with disability defined as mRS ≥ 3; time-to-disability analyzed via Kaplan–Meier and risk factors via Cox models; previously published morbidity nomograms were externally validated.
Cohort 332 patients (58.4% female; mean age 44.5); 19.8% familial CCM; 28.0% brainstem location; 38.3% presented with symptomatic hemorrhage (SH).
Recovery after first SH Among patients with SH who had no further SH and no surgery (n=48), disability (mRS ≥ 3) was 27.1% at diagnosis, improving to 6.2% at 1 year and 4.7% at 5 years, with most improvement in year 1.
Hemorrhage burden Over mean 6.6 years, 31.0% had ≥1 prospective SH and 14.5% had multiple prospective SHs; disability rose sharply with each SH (mRS ≥ 3: 2.3% with 0 SH up to 100% after 5 SHs in untreated follow-up).
Location risk Brainstem CCM carried substantially higher disability risk (18.8% at 5 years; 35.4% at 10 years) versus nonbrainstem locations (4.1% at 5 years; 7.5% at 10 years).
Predictors Multivariate predictors of disability included brainstem location, self-reported psychiatric disorder, and ≥2 SHs.
Nomogram validation Prior nomograms showed high specificity but limited sensitivity; AUC 0.687 for predicting mRS ≥ 2 and 0.783 for predicting mRS ≥ 3.
You must be logged in to post a comment.