title: Anti-glutamic acid decarboxylase limbic encephalitis without epilepsy evolving into dementia with cerebellar ataxia.
abstract: OBJECTIVE
  To expand the spectrum of the clinical presentation of anti-glutamic acid decarboxylase antibodies-related limbic encephalitis and to improve the recognition of this entity.
  
  
  METHODS
  Case study.
  
  
  METHODS
  University hospital.
  
  
  METHODS
  An 11-year-old-girl with progressive mood and behavioral disorder, speech impairment, and short-term memory impairment who manifested cerebellar ataxia with nystagmus during the disease course.
  
  
  METHODS
  Blood and cerebrospinal fluid analysis including autoantibodies, electroencephalography, brain and spinal magnetic resonance imaging, and cognitive and neuropsychological assessment were performed. High-dose methylprednisolone sodium succinate pulses, cycles of intravenous immunoglobulins, mycophenolate mofetil, and rituximab as well as antipsychotics and benzodiazepine were administered.
  
  
  RESULTS
  Diagnosis of anti-glutamic acid decarboxylase antibodies-related limbic encephalitis was made. The clinical features during the first months of disease included only mood, behavioral, and memory impairment. After 5 months, despite immunotherapies, cerebellar ataxia with nystagmus appeared with brain magnetic resonance imaging evidence of cerebral atrophy. No clinical or infraclinical seizures were recorded during follow-up.
  
  
  CONCLUSIONS
  Anti-glutamic acid decarboxylase antibodies-related limbic encephalitis can present with only behavioral or neuropsychological symptoms without any epileptic disorder. Moreover, cerebellar ataxia related to anti-glutamic acid decarboxylase antibodies can be observed in patients with limbic encephalitis during the disease course.
authors.affiliation: Units of Child Neuropsychiatry, Giannina Gaslini Institute, Genoa, Italy. marisol.mirabelli@gmail.com
authors.forename: Marisol
authors.initials: M
authors.lastname: Mirabelli-Badenier
authors.forename: Giovanni
authors.initials: G
authors.lastname: Morana
authors.forename: Francesca
authors.initials: F
authors.lastname: Pinto
authors.forename: Antonio
authors.initials: A
authors.lastname: Uccelli
authors.forename: Edvige
authors.initials: E
authors.lastname: Veneselli
authors.forename: Francesca Maria
authors.initials: FM
authors.lastname: Battaglia
authors.forename: Roberta
authors.initials: R
authors.lastname: Biancheri
authors.forename: Maria Giuseppina
authors.initials: MG
authors.lastname: Baglietto
authors.forename: Angela
authors.initials: A
authors.lastname: Vincent
authors.forename: Maria Margherita
authors.initials: MM
authors.lastname: Mancardi
mesh_terms: D002524:Cerebellar Ataxia / Q000175:diagnosis / Q000201:enzymology / Q000276:immunology*; D002648:Child; D003704:Dementia / Q000175:diagnosis / Q000201:enzymology / Q000276:immunology*; D004827:Epilepsy; D005260:Female; D005500:Follow-Up Studies; D005968:Glutamate Decarboxylase / Q000276:immunology*; D006801:Humans; D020363:Limbic Encephalitis / Q000175:diagnosis / Q000201:enzymology / Q000276:immunology*
chemical_list: D005968:Glutamate Decarboxylase