title: Encephalitis associated with glutamic acid decarboxylase autoantibodies in a child: a treatable condition?
abstract: OBJECTIVE
  To increase the recognition of glutamic acid decarboxylase autoantibodies-related encephalitis in childhood.
  
  
  METHODS
  Case report and review of the literature.
  
  
  METHODS
  A 6-year-old girl who had developed refractory seizures, developmental regression, and type 1 diabetes mellitus at age 25 months.
  
  
  METHODS
  Blood analysis, electroencephalogram, cerebral magnetic resonance imaging, positron emission tomography scan, lumbar puncture, and measurement of glutamic acid decarboxylase activity were performed. Treatment with repeated plasmapheresis and rituximab, with concomitant antiepileptic drugs, was administered.
  
  
  RESULTS
  Highly elevated titers of glutamic acid decarboxylase autoantibodies were found in the serum, as well as in the cerebrospinal fluid. Major clinical improvement in parallel with a decrease in the levels of serum and cerebrospinal fluid antibodies was observed with treatment.
  
  
  CONCLUSIONS
  Encephalitis associated with glutamic acid decarboxylase autoantibodies is a severe epileptic disorder that occurs in young children as well as adults. It may be partially reversible with aggressive immunomodulatory treatment, including plasmapheresis and rituximab. Studies are warranted to determine whether early treatment leads to complete remission.
authors.affiliation: Pediatric Neurology, Pediatric Specialties Service, University Hospital of Geneva, Switzerland. christian.korff@hcuge.ch
authors.forename: Christian M
authors.initials: CM
authors.lastname: Korff
authors.forename: Paloma
authors.initials: P
authors.lastname: Parvex
authors.forename: Laurent
authors.initials: L
authors.lastname: Cimasoni
authors.forename: Alexandra
authors.initials: A
authors.lastname: Wilhelm-Bals
authors.forename: Christiane S
authors.initials: CS
authors.lastname: Hampe
authors.forename: Valerie M
authors.initials: VM
authors.lastname: Schwitzgebel
authors.forename: Mélanie
authors.initials: M
authors.lastname: Michel
authors.forename: Claire-Anne
authors.initials: CA
authors.lastname: Siegrist
authors.forename: Patrice H
authors.initials: PH
authors.lastname: Lalive
authors.forename: Margitta
authors.initials: M
authors.lastname: Seeck
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000927:Anticonvulsants / Q000627:therapeutic use; D018501:Antirheumatic Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000096:biosynthesis / Q000097:blood* / Q000134:cerebrospinal fluid; D002648:Child; D004660:Encephalitis / Q000188:drug therapy* / Q000201:enzymology* / Q000276:immunology; D004827:Epilepsy / Q000188:drug therapy / Q000201:enzymology / Q000276:immunology; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology*; D006801:Humans; D010956:Plasmapheresis / Q000379:methods; D000069283:Rituximab; D005680:gamma-Aminobutyric Acid / Q000096:biosynthesis / Q000172:deficiency
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000927:Anticonvulsants; D018501:Antirheumatic Agents; D001323:Autoantibodies; D000069283:Rituximab; D005680:gamma-Aminobutyric Acid; D005968:Glutamate Decarboxylase