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Abstract Details

Oligosymptomatic anti-GABA A receptor encephalitis: a new diagnostic and therapeutic challenge
Autoimmune Neurology
P1 - Poster Session 1 (9:00 AM-5:00 PM)
040

To describe an oligosymptomatic case of anti-GABA A receptor encephalitis.

The antibodies against GABA A receptor are associated with a variety of severe neurological features, form status epilepticus to progressive encephalopathies. Sixty percent of the patients harbor a neoplasia, mainly thymoma. Brain MRI usually shows diffuse cortico-subcortical hyperintense (FLAIR) lesions. 
A 60-year old woman with a history of Chagas disease and ovarian cyst presented with headache and a fluctuating hemisensory syndrome also involving the face for the last 5 days. 
Brain MRI showed several supratentorial cortico-subcortical lesions that fluctuated with disappearance of some of them and appearance of new lesions. A few lesions showed mild gadolinium enhancement. EEG showed mild irritative changes in the right fronto-temporal region. CSF showed 10 lymphocytes/mm3 and some oligoclonal bands with pattern II. Extensive work-up for neoplasia, autoimmune and infectious conditions was negative. Immunohistochemistry study of serum and CSF on rodent brain showed the presence of antibodies against GABA A receptor. She spontaneously recovered, but in view of the results, was treated with a 5-day course if IV immunoglobulins. Her evolution so far (1 year of follow-up) has been benign, without any further relapses. 

GABA A receptor encephalitis may also have a more benign presentation, and should be considered in the presence of consistent MRI findings. The therapeutic approach in these cases remains challenging.

Authors/Disclosures
Iñigo Oyarzun Irazu, MD (Osakidetza)
PRESENTER
Dr. Oyarzun Irazu has nothing to disclose.
No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file
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Josep O. Dalmau, MD, PhD, FAAN Dr. Dalmau has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astellas Research Institute of America. Dr. Dalmau has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen Research & Development . Dr. Dalmau has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for 好色先生. An immediate family member of Dr. Dalmau has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Springer Nature. The institution of Dr. Dalmau has received research support from Sage Therapeutics. The institution of Dr. Dalmau has received research support from Edmond J.Safra Foundation . The institution of Dr. Dalmau has received research support from La Caixa Foundation. The institution of Dr. Dalmau has received research support from Spanish Ministry of Health (ISCIII). The institution of Dr. Dalmau has received research support from Euroimmun, Inc. Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care.
Francesc R. Graus, MD (IDIBAPS) Dr. Graus has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for MedLink. Dr. Graus has received intellectual property interests from a discovery or technology relating to health care.
Juan C. Garcia Monco Carra, MD, FAAN (Hospital De Basurto, Servicio De Neurologia) Dr. Garcia Monco Carra has nothing to disclose.