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Abstract Details

Status Epilepticus Suspected Autoimmune: Antibody Frequency and Main Clinical Features
Autoimmune Neurology
P2 - Poster Session 2 (8:00 AM-9:00 AM)
15-003

To characterize the frequency of neuronal surface antibodies (NS-Abs) and accompanying clinical features in patients who presented with status epilepticus (SE) suspected to be autoimmune.

Autoimmune encephalitis often associate with seizures or SE, which are resistant to antiepileptic drugs but potentially responsive to immunotherapy. SE can develop as initial manifestation of autoimmune encephalitis, but the confirmation with antibody determination may take several weeks resulting in diagnostic delays.
We retrospectively reviewed the clinical information of 102 patients (median age, 27 years [5-93 years]; 67 [65.7%] female) who presented with clinical features of SE suspected to be autoimmune. NS-Abs were examined in both serum and CSF (except 4 in either serum or CSF) obtained at symptom onset with previously reported rat brain immunohistochemistry and cell-based assays (NMDAR, LGI1, CASPR2, GABAaR, GABAbR, AMPAR, mGluR5, neurexin). MOG-Abs were only examined in patients clinically suspected to have acute disseminated encephalomyelitis or cortical encephalitis. We compared the clinical features of antibody-positive and negative patients.

34 patients (33.3%) had NS-Abs, including 29 NMDAR (1 of them with concurrent GABAbR and AMPAR, and another case with MOG); 2 GABAaR ; 1 GABAbR; 1 LGI1, and 1 against unknown antigens. Compared with NS-Ab-negative patients, NS-Ab-positive patients were more likely to be female (29/34 vs 38/68, p=0.0038), have more frequent accompanying psycho-behavioral or memory alterations before onset of SE (22/34 vs 10/68, p<0.0001), dyskinesias (29/34 vs 12/68, p<0.0001), CSF pleocytosis (29/34 vs 42/68, p=0.0215), oligoclonal bands (OCBs) (12/25 vs 3/56, p<0.0001), and tumor (15/34 vs 6/68, p<0.0001), and were less likely to have brain MRI abnormalities (12/34 vs 41/68, p=0.0214).    

In the current series NS-Abs were identified in 1/3 of patients with SE suspected to be autoimmune. Several clinical features such as psycho-behavioral or memory alterations, dyskinesias, CSF pleocytosis, OCBs, or tumor, are important clues that suggest the presence of NS-Abs.

Authors/Disclosures

PRESENTER 		No disclosure on file
Takahiro Iizuka, MD (Department of Neurology, Kitasato University School of Medicine) The institution of Dr. Iizuka has received research support from EUROIMMUN Japan Co., Ltd.
Juntaro Kaneko Juntaro Kaneko has nothing to disclose.
No disclosure on file
Atsushi Kaneko No disclosure on file
Eiji Kitamura Eiji Kitamura has nothing to disclose.
Naomi Kanazawa Naomi Kanazawa has nothing to disclose.
No disclosure on file
Josep O. Dalmau, MD, PhD, FÂ鶹´«Ã½Ó³»­ 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 Â鶹´«Ã½Ó³»­. 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.
Kazutoshi Nishiyama, MD, PhD Kazutoshi Nishiyama, MD, PhD has nothing to disclose.