Artikel
Multiple cerebral cavernous malformations – clinical course of confirmed, assumed and non-familial disease
Multiple zerebrale kavernöse Malformationen – Klinischer Verlauf von bestätigten, vermuteten und nicht-familiären Erkrankungen
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Veröffentlicht: | 25. Mai 2022 |
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Objective: Analyze and compare the natural course of confirmed, assumed and non-familial multiple cerebral cavernous malformation (CCM) disease over a 5-year period.
Methods: Our institutional database was screened for patients with CCM admitted between 2003 and 2020. Patients with complete magnetic resonance imaging dataset, evidence of multiple CCM, clinical baseline characteristics, and follow-up examination were included. Surgically treated individuals were censored after CCM removal. Patients were separated into confirmed familial cases, assumed familial cases or non-familial cases of CCM. Kaplan–Meier and Cox regression analyses were performed to determine the cumulative 5-year risk for hemorrhage and recurrent hemorrhage.
Results: 238 patients with multiple CCM were analyzed. 90 individuals had a confirmed FCCM disease, 115 an assumed FCCM, and 33 were allocated to the non-FCCM group. Univariate Cox regression analysis identified intracerebral hemorrhage (ICH) as mode of presentation (P=0.001; HR, 4.66; 95% CI, 1.80-12.03) as a predictor for occurrence of recurrent hemorrhage during the 5-year follow-up (FU). The cumulative 5-year risk of (re)bleeding was 21.6% for the entire cohort, 30.7% for patients with ICH at diagnosis, 22.1% for those patients with a confirmed diagnosis of FCCM, 23.5% for those with an assumed FCCM, and 21% for the non-FCCM cases.
Conclusion: FCCM patients with ICH at diagnosis are prone to develop rebleeding. During untreated 5-year FU, FCCM patients and patients with sporadic multiple CCM reveal an almost equal susceptibility for (re)hemorrhage. Moreover, confirmed, assumed and non-FCCM patients showed an equal cumulative 5-year risk of symptomatic ICH. The probability of hemorrhage tends to increase over time, particularly in cases with ICH at presentation.