期刊
EPILEPSIA
卷 54, 期 3, 页码 543-550出版社
WILEY-BLACKWELL
DOI: 10.1111/epi.12042
关键词
Rasmussen encephalitis; Incidence; Treatment
资金
- Astellas Pharma GmbH, Munchen, Germany
- Octapharma GmbH, Lingen, Germany
- Eisai (Germany)
- UCB (Germany)
- Desitin (Germany)
- Grifols (Germany)
- VIDI scheme [NOW-ZonMw 017.106.370]
- Sopohia Children's Hospital Research Foundation [SSWO-2009-628]
- Pfizer (Germany)
- government entity Deutsche Forschungsgemeinschaft
- University of Bonn
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 >12years at disease onset: 8%) led to study exit. Untreated patients served as a historical control group. Key Findings: Over 6.3years, 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 2.4cases/107peopleage 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.
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