Health utilities and quality of life in individuals with tuberous sclerosis complex (TSC) who experience epileptic seizures: A web-based survey

2019 ◽  
Vol 92 ◽  
pp. 213-220 ◽  
Author(s):  
Theo Tritton ◽  
Bryan Bennett ◽  
Elaine Brohan ◽  
Laura Grant ◽  
Alison Cooper ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Epileptic Seizures ◽  
Web Based ◽  
Health Utilities

scholarly journals Quality of life in patients with Tuberous Sclerosis Complex (TSC)

2019 ◽  
Vol 23 (6) ◽  
pp. 801-807 ◽  
Author(s):  
Sam Amin ◽  
Andrew A. Mallick ◽  
Andrew Lux ◽  
Finbar O'Callaghan
Keyword(s):  
Quality Of Life ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex

scholarly journals Epilepsy, impaired functioning, and quality of life in patients with tuberous sclerosis complex

2019 ◽  
Vol 4 (4) ◽  
pp. 581-592 ◽  
Author(s):  
Menno Vergeer ◽  
Wendela L. Ranitz‐Greven ◽  
Maureen P. Neary ◽  
Raluca Ionescu‐Ittu ◽  
Bruno Emond ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex

Quality of life following epilepsy surgery for children with tuberous sclerosis complex

2011 ◽  
Vol 20 (3) ◽  
pp. 561-565 ◽  
Author(s):  
Jonathan Roth ◽  
Adeolu Olasunkanmi ◽  
William S. MacAllister ◽  
Emily Weil ◽  
Catherine C. Uy ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Tuberous Sclerosis ◽  
Epilepsy Surgery ◽  
Tuberous Sclerosis Complex

scholarly journals Sirolimus Ointment for Facial Angiofibromas in Individuals with Tuberous Sclerosis Complex

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
S. Amin ◽  
A. Lux ◽  
A. Khan ◽  
F. O’Callaghan
Keyword(s):  
Quality Of Life ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Positive Impact ◽  
Severity Index ◽  
Recurrent Bleeding ◽  
Facial Disfigurement ◽  
Psychosocial Domain ◽  
The Impact

Background. Facial angiofibromas affect most patients with tuberous sclerosis complex. They tend to progress, can cause recurrent bleeding and facial disfigurement, and have significant psychological effects. We reviewed the effectiveness and safety of topical sirolimus ointment 0.1%. We also assessed the effect of treatment on quality of life. Methods. We report our experience in using sirolimus ointment in 14 patients with TSC (9 children and 5 adults). The impact of sirolimus ointment was monitored with digital photography, dermatological review using a validated Facial Angiofibroma Severity Index (FASI), and quality of life assessments using the questionnaires PedsQL for children and SF36 for adults. Results. The FASI scores were improved in 12/14 cases after six months’ treatment, and improvement was more likely in children (median FASI scores of improvement after treatment were 3 points for children and 1 for adults). Proxy-reported PedsQL scores for the total psychosocial domain improved significantly in the children in the cohort with treatment. Conclusions. Sirolimus ointment 0.1% administered once a day was effective in treating facial angiofibromas. It appears to be safe and well tolerated and to have a positive impact on patients’ quality of life. It appeared to be most beneficial when started in childhood.

scholarly journals Burden of Illness and Quality of Life in Tuberous Sclerosis Complex: Findings From the TOSCA Study

2020 ◽  
Vol 11 ◽  
Author(s):  
Anna C. Jansen ◽  
Stephanie Vanclooster ◽  
Petrus J. de Vries ◽  
Carla Fladrowski ◽  
Guillaume Beaure d'Augères ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Burden Of Illness

scholarly journals Resective epilepsy surgery in tuberous sclerosis complex: a nationwide multicentre retrospective study from China

Brain ◽  
2020 ◽  
Vol 143 (2) ◽  
pp. 570-581 ◽  
Author(s):  
Shiyong Liu ◽  
Tao Yu ◽  
Yuguang Guan ◽  
Kai Zhang ◽  
Ping Ding ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Tuberous Sclerosis ◽  
Epilepsy Surgery ◽  
Tuberous Sclerosis Complex ◽  
Intelligence Quotient ◽  
Influence Factors ◽  
Seizure Freedom ◽  
Postoperative Seizure

Abstract At least 50% of patients with tuberous sclerosis complex present with intractable epilepsy; for these patients, resective surgery is a treatment option. Here, we report a nationwide multicentre retrospective study and analyse the long-term seizure and neuropsychological outcomes of epilepsy surgery in patients with tuberous sclerosis complex. There were 364 patients who underwent epilepsy surgery in the study. Patients’ clinical data, postoperative seizure outcomes at 1-, 4-, and 10-year follow-ups, preoperative and postoperative intelligence quotients, and quality of life at 1-year follow-up were collected. The patients’ ages at surgery were 10.35 ± 7.70 years (range: 0.5–47). The percentage of postoperative seizure freedom was 71% (258/364) at 1-year, 60% (118/196) at 4-year, and 51% (36/71) at 10-year follow-up. Influence factors of postoperative seizure freedom were the total removal of epileptogenic tubers and the presence of outstanding tuber on MRI at 1- and 4-year follow-ups. Furthermore, monthly seizure (versus daily seizure) was also a positive influence factor for postoperative seizure freedom at 1-year follow-up. The presence of an outstanding tuber on MRI was the only factor influencing seizure freedom at 10-year follow-up. Postoperative quality of life and intelligence quotient improvements were found in 43% (112/262) and 28% (67/242) of patients, respectively. Influence factors of postoperative quality of life and intelligence quotient improvement were postoperative seizure freedom and preoperative low intelligence quotient. The percentage of seizure freedom in the tuberectomy group was significantly lower compared to the tuberectomy plus and lobectomy groups at 1- and 4-year follow-ups. In conclusion, this study, the largest nationwide multi-centre study on resective epilepsy surgery, resulted in improved seizure outcomes and quality of life and intelligence quotient improvements in patients with tuberous sclerosis complex. Seizure freedom was often achieved in patients with an outstanding tuber on MRI, total removal of epileptogenic tubers, and tuberectomy plus. Quality of life and intelligence quotient improvements were frequently observed in patients with postoperative seizure freedom and preoperative low intelligence quotient.

scholarly journals SP020HEALTH-RELATED QUALITY OF LIFE FOR PATIENTS WITH TUBEROUS SCLEROSIS COMPLEX AND ASSOCIATED MANIFESTATIONS

2017 ◽  
Vol 32 (suppl_3) ◽  
pp. iii110-iii110
Author(s):  
Maureen Neary ◽  
Francis Vekeman ◽  
Raluca Ionescu-Ittu ◽  
Bruno Emond ◽  
Mei Duh ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Related Quality

Percutaneous microwave ablation of renal angiomyolipomas in tuberous sclerosis complex to improve the quality of life: preliminary experience in an Italian center

2018 ◽  
Vol 124 (3) ◽  
pp. 176-183 ◽  
Author(s):  
Anna Maria Ierardi ◽  
Mario Petrillo ◽  
Andrea Coppola ◽  
Salvatore Alessio Angileri ◽  
Andrea Galassi ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Microwave Ablation ◽  
Percutaneous Microwave Ablation

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

2021 ◽  
Vol 3 (1) ◽  
Author(s):  
Johann Philipp Zöllner ◽  
Nadine Conradi ◽  
Matthias Sauter ◽  
Markus Knuf ◽  
Susanne Knake ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Regression Analysis ◽  
Adverse Events ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Neurological Disorders ◽  
Organ Manifestations ◽  
Neuropsychiatric Manifestations ◽  
Active Epilepsy

Abstract 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.

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