Characteristics and healthcare situation of adult patients with tuberous sclerosis complex in German epilepsy centers

Hamer H, Pfaefflin M, Baier H, Boesebeck F, Franz M, Holtkamp M, Kurlemann G, May T, Mayer T, Metzner M, Steinhoff BJ, Stodieck S, Straub HB, Weber YG, Brandt C (2018)

Publication Type: Journal article

Publication year: 2018

Journal

Epilepsy and Behavior Elsevier

Book Volume: 82

Pages Range: 64-67

DOI: 10.1016/j.yebeh.2018.03.006

Abstract

The objective of the present study was to collect systematic data on the care of adult patients with tuberous sclerosis complex (TSC) in German epilepsy centers, to describe the characteristics of patients in this age group, and to clarify whether and how the recommended interdisciplinary care is implemented.This retrospective survey involved 12 major epilepsy centers in Germany. Aggregated data were collected based on an electronic questionnaire that addressed the sociodemographic data, characteristics of the epilepsy syndromes, and general healthcare setting of adult patients with TSC.The survey included 262 patients (mean age: 36.2±9.0years) with TSC, most of whom were reported to live in either a home for persons with a disability (37.0%), a residential care home (6.9%), or with their parents (31.1%). A further 13.0% were self-sustaining, and 8.8% were living with a partner. Most patients presented with focal (49.6%) or multifocal (33.2%) epilepsy, with complex partial, dialeptic, and automotor seizures in 66% of patients and generalized tonic-clonic seizures in 63%. Drug-refractory epilepsy was seen in 78.2% of patients, and 17.6% were seizure-free at the time of the survey. Of the 262 patients, presurgical diagnostics were performed in 27% and epilepsy surgery in 9%, which rendered 50% of these patients seizure-free. Renal screening had been performed in 56.1% within the last three years and was scheduled to be performed in 58.0%. Cases of renal angiomyolipoma were present in 46.9% of the patients. Dermatologic and pulmonary screenings were known to be planned for only few patients.Despite TSC being a multisystem disorder causing considerable impairment, every fifth adult patient is self-sustaining or living with a partner. In clinical practice, uncontrolled epilepsy and renal angiomyolipoma are of major importance in adult patients with TSC. Most patients suffer from focal or multifocal epilepsy, but epilepsy surgery is performed in less than 10% of these patients. Interdisciplinary TSC centers may help to optimize the management of patients with TSC regardless of age and ensure early and adequate treatment that also considers the advances in new therapeutic options.

Authors with CRIS profile

Hajo Hamer Professur für Epileptologie

Involved external institutions

Gesellschaft für Epilepsieforschung e.V. DE Germany (DE) Epilepsie-Zentrum Bodensee DE Germany (DE) Agaplesion Diakonieklinikum Rotenburg DE Germany (DE) Novartis AG CH Switzerland (CH) Evangelisches Krankenhaus Königin Elisabeth Herzberge DE Germany (DE) Universitätsklinikum Münster DE Germany (DE) Kleinwachau - Sächsisches Epilepsiezentrum Radeberg gGmbH DE Germany (DE) Epilepsiezentrum Kork DE Germany (DE) Evangelisches Krankenhaus Alsterdorf DE Germany (DE) Epilepsieklinik Tabor DE Germany (DE) Eberhard Karls Universität Tübingen DE Germany (DE) Krankenhaus Mara DE Germany (DE)

How to cite

APA:

Hamer, H., Pfaefflin, M., Baier, H., Boesebeck, F., Franz, M., Holtkamp, M.,... Brandt, C. (2018). Characteristics and healthcare situation of adult patients with tuberous sclerosis complex in German epilepsy centers. Epilepsy and Behavior, 82, 64-67. https://dx.doi.org/10.1016/j.yebeh.2018.03.006

MLA:

Hamer, Hajo, et al. "Characteristics and healthcare situation of adult patients with tuberous sclerosis complex in German epilepsy centers." Epilepsy and Behavior 82 (2018): 64-67.

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