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. 2022 May;29(5):1427-1434.
doi: 10.1111/ene.15253. Epub 2022 Feb 3.

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

Alejandro N Santos  1 , Laurèl Rauschenbach  1 , Dino Saban  1 , Bixia Chen  1 , Marvin Darkwah Oppong  1 , Annika Herten  1 , Hanah Hadice Gull  1 , Christoph Rieß  1 , Cornelius Deuschl  2 , Börge Schmidt  3 , Ramazan Jabbarli  1 , Karsten H Wrede  1 , Yuan Zhu  1 , Benedikt Frank  4 , Ulrich Sure  1 , Philipp Dammann  1
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Multiple cerebral cavernous malformations: Clinical course of confirmed, assumed and non-familial disease

Alejandro N Santos et al. Eur J Neurol. 2022 May.

Abstract

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.

Keywords: CCM; cavernous angioma; familial; multiple cerebral cavernous malformations; natural course; risk factors.

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References

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