Multiple cerebral cavernous malformations: Clinical course of confirmed, assumed and non‐familial disease

التفاصيل البيبلوغرافية
العنوان: Multiple cerebral cavernous malformations: Clinical course of confirmed, assumed and non‐familial disease
المؤلفون: Alejandro N. Santos, Laurèl Rauschenbach, Dino Saban, Bixia Chen, Marvin Darkwah Oppong, Annika Herten, Hanah Hadice Gull, Christoph Rieß, Cornelius Deuschl, Börge Schmidt, Ramazan Jabbarli, Karsten H. Wrede, Yuan Zhu, Benedikt Frank, Ulrich Sure, Philipp Dammann
المصدر: European Journal of Neurology. 29:1427-1434
بيانات النشر: Wiley, 2022.
سنة النشر: 2022
مصطلحات موضوعية: Hemangioma, Cavernous, Central Nervous System, Neurology, Medizin, Humans, Neurology (clinical), Magnetic Resonance Imaging, Cerebral Hemorrhage
الوصف: in press Background and Purpose Analyze and compare the natural course of confirmed familial cerebral cavernous malformation (FCCM), assumed FCCM 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. Patients were separated into confirmed familial cases, assumed familial cases or non-familial multiple cavernous malformations. Kaplan-Meier and Cox regression analyses were performed to determine the cumulative 5-year risk for hemorrhage and recurrent hemorrhage. Results A total of 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) 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. Conclusions 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.
تدمد: 1468-1331
1351-5101
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d21d7f1fac5fa3a0071d5efb344a2b56
https://doi.org/10.1111/ene.15253
حقوق: OPEN
رقم الأكسشن: edsair.doi.dedup.....d21d7f1fac5fa3a0071d5efb344a2b56
قاعدة البيانات: OpenAIRE