Abstract Details

Title Alpha-Amino-3-Hydroxy-5-Methyl-4-Isoxazoleproprionic Acid (AMPA) Receptor Mediated Limbic Encephalitis in a Fourteen-Year-Old

Topic Behavioral Neurology

Presentation(s) P05 - (-)

Poster/Presentation
Number 114

Objective

Background BACKGROUND: AMPA receptor mediated limbic encephalitis is an inflammatory, immune mediated disorder characterized by an acute to sub-acute disturbance in mood, behavior, and cognition. It is a relatively less commonly recognized entity and has previously been reported in middle-aged to elderly patients, mostly female and over the age of fifty. We present the case of a fourteen-year-old female with AMPA receptor mediated encephalitis.

Design/Methods DESIGN/METHODS: Case report from a tertiary care medical center.

Results RESULTS: A fourteen year old previously healthy honor roll student presented to the hospital after her family noted mental status deterioration over a three-week period. They described specific instances of cognitive problems including short-term memory loss, confusion, and apathy. On examination, she demonstrated a flat affect, inattention, and responded to questions inconsistently. She made paraphasic errors and her gait was ataxic. There were no adventitious movements. Routine serological and cerebrospinal fluid (CSF) studies were unremarkable. CSF sample was sent to an out-of-state laboratory for further comprehensive molecular testing. Magnetic resonance imaging (MRI) of the brain was significant for faint areas of cortical signal abnormality in the right frontal parasaggital and left peri-insular regions and MRI of the abdomen and pelvis revealed no neoplasm. Empiric intravenous immunoglobulin (IVIG) therapy was initiated while awaiting CSF study results. The patient's language, attention, and ataxia did marginally improve after treatment and she returned to school. Six weeks after initial presentation, AMPA receptor antibody in the CSF was reported positive.

Conclusions CONCLUSIONS: Although AMPA receptor encephalitis has previously been described in middle-aged to elderly patients, this case demonstrates that it may be considered in younger patients who present with a limbic encephalitis picture.

Authors/Disclosures
Shouri Lahiri, MD (Cedars-Sinai Medical Center) PRESENTER	Dr. Lahiri has received personal compensation in the range of $500-$4,999 for serving as a Consultant for AstraZeneca. Dr. Lahiri has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for medicolegal consultations.. Dr. Lahiri has stock in Prometheus. The institution of Dr. Lahiri has received research support from National Institutes of Health. The institution of Dr. Lahiri has received research support from F. Widjaja Foundation.
Susan E. Duberstein Coad, MD	Dr. Duberstein Coad has nothing to disclose.
Stuart D. Cook, MD, FAAN (Rutgers)	No disclosure on file

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