0
Observation |

Anti–Glutamic Acid Decarboxylase Limbic Encephalitis Without Epilepsy Evolving Into Dementia With Cerebellar Ataxia

Marisol Mirabelli-Badenier, MD; Giovanni Morana, MD; Francesca Pinto, MD; Antonio Uccelli, MD; Edvige Veneselli, MD, PhD; Francesca Maria Battaglia, PhD; Roberta Biancheri, MD, PhD; Maria Giuseppina Baglietto, MD, PhD; Angela Vincent, FRCP; Maria Margherita Mancardi, MD, PhD
Arch Neurol. 2012;69(8):1064-1066. doi:10.1001/archneurol.2012.234.
Text Size: A A A
Published online

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.

Figures in this Article

Sign In to Access Full Content

Don't have Access?

Register and get free email Table of Contents alerts, saved searches, PowerPoint downloads, CME quizzes, and more

Subscribe for full-text access to content from 1998 forward and a host of useful features

Activate your current subscription (AMA members and current subscribers)

Purchase Online Access to this article for 24 hours

Figures

Place holder to copy figure label and caption
Grahic Jump Location

Figure. Brain magnetic resonance imaging. A, Coronal fluid-attenuated inversion recovery imaging performed at admission shows hyperintensity (arrows) involving the cortical-subcortical mesial temporal region bilaterally, more extensive on the left, where the signal abnormalities also affect the subinsular region. B, Coronal T2-weighted imaging performed 4 months later demonstrates severe atrophic changes of the affected regions with ex vacuo dilatation of the ventricular system and adjacent subarachnoid spaces.

Tables

Interactive Graphics

Video

Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

References

Correspondence

CME
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s “Cited By” API will populate this tab (http://www.crossref.org/citedby.html).
Submit a Comment

Some tools below are only available to our subscribers or users with an online account.

Sign In to Access Full Content

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Topics
Jobs