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Rasmussen encephalitis: Incidence and course under randomized therapy with tacrolimus or intravenous immunoglobulins |
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| Authors: | Christian G Bien Henning Tiemeier Robert Sassen Stefan Kuczaty Horst Urbach Marec von Lehe Albert J Becker Thomas Bast Peter Herkenrath Michael Karenfort Bernd Kruse Gerhard Kurlemann Sabine Rona Susanne Schubert‐Bast Silvia Vieker Stefan Vlaho Bernd Wilken Christian E Elger |
| Institution: | 1. Department of Epileptology, University of Bonn Medical Center, Bonn, Germany;2. Department of Epidemiology, Erasmus Medical Center, Rotterdam, The Netherlands;3. Departments of Radiology/Neuroradiology;4. Neurosurgery;5. Neuropathology, University of Bonn Medical Center, Bonn, Germany;6. Department of General Pediatrics, University Children’s Hospital, Heidelberg, Germany;7. Department of Pediatric Neurology with Social Pediatric Center, University of Cologne, Cologne, Germany;8. Department of Neuropediatrics, University of G?ttingen, G?ttingen, Germany;9. Department of Pediatrics, University Clinic Hamburg‐Eppendorf, Hamburg, Germany;10. Department of General Pediatrics, Neuropediatrics, University Children’s Hospital Muenster, Muenster, Germany;11. Department of Neurosurgery, Epilepsy Center, University of Freiburg, Freiburg, Germany;12. Clinic for Child and Adolescent Medicine, Evangelisches Krankenhaus Bielefeld, Bielefeld, Germany;13. Department of Child Neurology, Center of Pediatrics, Clinic I, J.W. Goethe University, Frankfurt/Main, Germany;14. Department of Pediatric Neurology, Hospital Kassel, Kassel, Germany |
| Abstract: | Purpose: Rasmussen encephalitis (RE) leads to progressive tissue and function loss of one brain hemisphere and often intractable epilepsy. This is the first randomized prospective treatment trial in RE. Methods: Germany‐wide, patients with suspected recent‐onset RE were recruited and if eligible randomized to tacrolimus or intravenous immunoglobulins (IVIGs). A loss of motor function or hemispheric volume by ≥15% (in patients >12 years at disease onset: ≥8%) led to study exit. Untreated patients served as a historical control group. Key Findings: Over 6.3 years, 21 patients with recent‐onset RE were identified. Sixteen were randomized to tacrolimus (n = 9) or IVIG (n = 7). Immunotreated patients had a longer “survival” than the historical controls. Neither treatment was more efficacious than the other. Two tacrolimus patients experienced serious adverse events. No immunotreated but several untreated patients developed intractable epilepsy. No patient with refractory epilepsy became treatment‐responsive under immunotherapy. Significance: The countrywide incidence rate of diagnosed RE is estimated as 2.4 cases/107 people ≤ age 18/year. Treatment with tacrolimus or IVIG may slow down tissue and function loss and prevent development of intractable epilepsy. However, immunotherapy may “arrest” patients in a dilemma state of pharmacoresistant epilepsy but too good function to be offered functional hemispherectomy. These compounds may therefore contribute to the therapeutic armamentarium for RE patients without difficult‐to‐treat epilepsies. |
| Keywords: | Rasmussen encephalitis Incidence Treatment |