Anti-glutamic acid decarboxylase limbic encephalitis without epilepsy evolving into dementia with cerebellar ataxia

Marisol Mirabelli-Badenier, Giovanni Morana, Francesca Pinto, Antonio Uccelli, Edvige Veneselli, Francesca Maria Battaglia, Roberta Biancheri, Maria Giuseppina Baglietto, Angela Vincent, Maria Margherita Mancardi

Research output: Contribution to journalArticle

Abstract

Objectives: 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. Design : Case study. Setting: University hospital. Patient: An 11-year-old-girl with progressive mood and behavioral disorder, speech impairment, and shortterm memory impairment who manifested cerebellar ataxia with nystagmus during the disease course. Interventions: 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 patientswith limbic encephalitis during the disease course.

Original languageEnglish
Pages (from-to)1064-1066
Number of pages3
JournalArchives of Neurology
Volume69
Issue number8
DOIs
Publication statusPublished - Aug 2012

ASJC Scopus subject areas

  • Clinical Neurology
  • Arts and Humanities (miscellaneous)

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    Mirabelli-Badenier, M., Morana, G., Pinto, F., Uccelli, A., Veneselli, E., Battaglia, F. M., Biancheri, R., Baglietto, M. G., Vincent, A., & Mancardi, M. M. (2012). Anti-glutamic acid decarboxylase limbic encephalitis without epilepsy evolving into dementia with cerebellar ataxia. Archives of Neurology, 69(8), 1064-1066. https://doi.org/10.1001/archneurol.2012.234