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

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 short-term 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 patients with limbic encephalitis during the disease course.

• Authors: Marisol Mirabelli-Badenier, Giovanni Morana, Francesca Pinto, Antonio Uccelli, Edvige Veneselli, Francesca Maria Battaglia, Roberta Biancheri, Maria Giuseppina Baglietto, Angela Vincent, Maria Margherita Mancardi
• Journal: Archives of neurology (Arch Neurol) Vol. 69 Issue 8 Pg. 1064-6 (Aug 2012) ISSN: 1538-3687 [Electronic] United States
• PMID: 22507885 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Glutamate Decarboxylase

Topics

• Cerebellar Ataxia (diagnosis, enzymology, immunology)
• Child
• Dementia (diagnosis, enzymology, immunology)
• Epilepsy
• Female
• Follow-Up Studies
• Glutamate Decarboxylase (immunology)
• Humans
• Limbic Encephalitis (diagnosis, enzymology, immunology)