To estimate health related quality of life (HRQOL) in patients with untreated cavernous malformation of the CNS (CCM).
We performed a cross-sectional observational study on patients with CCM admitted to our department from 01.11.2017-10.01.2020 using standardized interviews (short-form -36 questionnaire, hospital anxiety and depression score (HADS-A/D), CCM-perception questionnaire). Including criteria was diagnosis of an untreated CCM and information about the diagnosis in a specialized CCM consultation. HRQOL data was analyzed and compared to German normal population. Uni- and multivariate analyses were carried out to identify variables with impact on outcome.
219 (93%) of 229 eligible patients were included. Mean age was 46.3 ±14.7 (18-86) years, 136 (62%) were female. 98 (45%) patients presented with symptomatic hemorrhage and 17 (8%) with repetitive SH. 92 (42%) patients were asymptomatic. 37 (17%) suffered from cavernoma-related epilepsy. 28 patients (13%) suffered from familial CCM. Patients showed significantly decreased component scores and subdomain scores compared to normal population with effects ranging from small to large. This accounted largely also for asymptomatic patients (except for physical component score and main physical subdomains). Multivariate regression analysis confirmed impact of functional impairment on physical component score. HADS-A was significantly increased. HADS-A/D strongly correlated with mental component score and individual perception of the CCM.
Patients with the diagnosis of a CCM showed decreased HRQOL compared to normal population even when not suffering functional impairment or neurological symptoms. Our data may function as benchmark in evaluation of different (future) management strategies.

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