Quality of life and its predictors in adults with tuberous sclerosis complex (TSC): a multicentre cohort study from Germany

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dc.contributor.authorZöllner, Johann P.
dc.contributor.authorConradi, Nadine
dc.contributor.authorSauter, Matthias
dc.contributor.authorKnuf, Markus
dc.contributor.authorKnake, Susanne
dc.contributor.authorKurlemann, Gerhard
dc.contributor.authorMayer, Thomas
dc.contributor.authorHertzberg, Christoph
dc.contributor.authorBertsche, Astrid
dc.contributor.authorImmisch, Ilka
dc.contributor.authorKlein, Karl M.
dc.contributor.authorMarquard, Klaus
dc.contributor.authorMeyer, Sascha
dc.contributor.authorNoda, Anna H.
dc.contributor.authorvon Podewils, Felix
dc.contributor.authorSchäfer, Hannah
dc.contributor.authorThiels, Charlotte
dc.contributor.authorZukunft, Bianca
dc.contributor.authorSchubert-Bast, Susanne
dc.contributor.authorGrau, Janina
dc.contributor.authorWillems, Laurent M.
dc.contributor.authorRosenow, Felix
dc.contributor.authorReese, Jens-Peter
dc.contributor.authorStrzelczyk, Adam
dc.date.accessioned2021-07-04T00:03:37Z
dc.date.available2021-07-04T00:03:37Z
dc.date.issued2021-06-28
dc.date.updated2021-07-04T00:03:37Z
dc.description.abstractAbstract Background Tuberous sclerosis complex (TSC) is a monogenetic, multisystemic disease characterised by the formation of benign tumours that can affect almost all organs, caused by pathogenic variations in TSC1 or TSC2. In this multicentre study from Germany, we investigated the influence of sociodemographic, clinical, and therapeutic factors on quality of life (QoL) among individuals with TSC. Methods We assessed sociodemographic and clinical characteristics and QoL among adults with TSC throughout Germany using a validated, three-month, retrospective questionnaire. We examined predictors of health-related QoL (HRQoL) using multiple linear regression analysis and compared the QoL among patients with TSC with QoL among patients with other chronic neurological disorders. Results We enrolled 121 adults with TSC (mean age: 31.0 ± 10.5 years; range: 18–61 years, 45.5% [n = 55] women). Unemployment, a higher grade of disability, a higher number of organ manifestations, the presence of neuropsychiatric manifestations or active epilepsy, and a higher burden of therapy-related adverse events were associated with worse QoL, as measured by two QoL instruments (EuroQoL-5 dimensions [EQ-5D] and Quality of Life in Epilepsy Patients [QOLIE-31]). Neuropsychiatric and structural nervous system manifestations, the number of affected organs, and therapy-related adverse events were also associated with higher depression, as measured by the Neurological Disorders Depression Inventory for Epilepsy (NDDI-E). In multiple regression analysis, more severe therapy-related adverse events (large effect, p < 0.001), active epilepsy (large effect, p < 0.001), and neuropsychiatric manifestations (medium effect, p = 0.003) were independently associated with worse HRQoL, explaining 65% of the variance (p < 0.001). The HRQoL among patients with active TSC-associated epilepsy was worse than that among patients with drug-refractory mesial temporal lobe epilepsy (p < 0.001), and the generic QoL among patients with more than three TSC organ manifestations was similar to those of patients with severe migraine and uncontrolled asthma. Conclusions Active epilepsy, neuropsychiatric manifestations (such as anxiety and depression), and therapy-related adverse events are important independent predictors of worse quality of life among adults with TSC. Generic quality of life in TSC with several manifestations is similar to uncontrolled severe chronic diseases and significantly negatively correlates with TSC severity. Trial registration DRKS, DRKS00016045 . Registered 01 March 2019.
dc.identifier.citationNeurological Research and Practice. 2021 Jun 28;3(1):35
dc.identifier.doihttps://doi.org/10.1186/s42466-021-00130-3
dc.identifier.urihttp://hdl.handle.net/1880/113585
dc.identifier.urihttps://doi.org/10.11575/PRISM/45549
dc.language.rfc3066en
dc.rights.holderThe Author(s)
dc.titleQuality of life and its predictors in adults with tuberous sclerosis complex (TSC): a multicentre cohort study from Germany
dc.typeJournal Article
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