title: Rasmussen encephalitis: incidence and course under randomized therapy with tacrolimus or intravenous immunoglobulins.
abstract: OBJECTIVE
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Epileptology, University of Bonn Medical Center, Bonn, Germany. christian.bien@gmx.de
authors.forename: Christian G
authors.initials: CG
authors.lastname: Bien
authors.forename: Henning
authors.initials: H
authors.lastname: Tiemeier
authors.forename: Robert
authors.initials: R
authors.lastname: Sassen
authors.forename: Stefan
authors.initials: S
authors.lastname: Kuczaty
authors.forename: Horst
authors.initials: H
authors.lastname: Urbach
authors.forename: Marec
authors.initials: M
authors.lastname: von Lehe
authors.forename: Albert J
authors.initials: AJ
authors.lastname: Becker
authors.forename: Thomas
authors.initials: T
authors.lastname: Bast
authors.forename: Peter
authors.initials: P
authors.lastname: Herkenrath
authors.forename: Michael
authors.initials: M
authors.lastname: Karenfort
authors.forename: Bernd
authors.initials: B
authors.lastname: Kruse
authors.forename: Gerhard
authors.initials: G
authors.lastname: Kurlemann
authors.forename: Sabine
authors.initials: S
authors.lastname: Rona
authors.forename: Susanne
authors.initials: S
authors.lastname: Schubert-Bast
authors.forename: Silvia
authors.initials: S
authors.lastname: Vieker
authors.forename: Stefan
authors.initials: S
authors.lastname: Vlaho
authors.forename: Bernd
authors.initials: B
authors.lastname: Wilken
authors.forename: Christian E
authors.initials: CE
authors.lastname: Elger
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004660:Encephalitis / Q000188:drug therapy* / Q000453:epidemiology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D015994:Incidence; D008137:Longitudinal Studies; D008297:Male; D011446:Prospective Studies; D012189:Retrospective Studies; D016559:Tacrolimus / Q000008:administration & dosage*; D055815:Young Adult
chemical_list: D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D016559:Tacrolimus