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Abstract Details

Atypical Clinical Manifestations and Overlapping Immunities associated with NMDA Receptor Antibodies
Autoimmune Neurology
S11 - Autoimmune CNS Inflammatory Disorders: Clinical Advances (4:25 PM-4:36 PM)
006

Six major groups of symptoms of anti-NMDAR encephalitis include, 1) abnormal behaviour or cognitive dysfunction (BCD), 2) speech dysfunction (SD), 3) seizures, 4) movement disorders (MD), 5) decreased level of consciousness (DC), and 6) autonomic dysfunction or central hypoventilation (AD/CH). According to recently published criteria, a definite diagnosis of anti-NMDAR encephalitis can be made with one or more of the 6 major groups of symptoms in association with IgG GluN1-antibodies (NMDAR-antibodies) in CSF. The frequency of clinically atypical cases associated with these antibodies is unclear.

To report atypical clinical manifestations associated with NMDA receptor (NMDAR) antibodies.

We reviewed the clinical information of 43 patients (median age 27 years [12-51 years], 33 [76.7%] female) with NMDAR-antibodies in CSF and/or serum detected with cell-based assays and rat brain immunohistochemistry. Typical cases were defined as those with 4 or more of the 6 major groups of symptoms, while atypical cases as those with 3 or less.

In the 43 patients, the median number of major groups of symptoms were 6 (range, 0-6): BCD (n=40), seizures (n=37), SD (n=35), DC (n=35), MD (n=34) and AD/CH (n=30). The clinical features were divided into 2 groups: typical (n=34 patients) and atypical (n=9, 20.9%). Comparison of both groups showed no difference in age of onset, gender, prodromal fever or headache, CSF pleocytosis, oligoclonal bands, or tumor association; however, ventilatory support was less frequently used in atypical cases (1/9 vs 25/34, p=0.0012). The symptoms in atypical cases included isolated seizures (n=3, 1 with MOG-antibodies), isolated psychosis (n=2), multifocal demyelinating syndrome (n=2), meningoencephalitis with leptomeningeal enhancement (n=1, with MOG-antibodies), and autoimmune post-herpes simplex encephalitis (n=1).

In this cohort, 3 of 43 (7%) patients with NMDAR-antibodies, including 2 with multifocal demyelination and 1 meningoencephalitis with overlapping MOG-antibodies, showed atypical clinical features not included in the criteria of anti-NMDAR encephalitis.

Authors/Disclosures
Atsushi Kaneko
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.
No disclosure on file
Juntaro Kaneko Juntaro Kaneko 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
No disclosure on file
No disclosure on file
No disclosure on file
Eiji Kitamura Eiji Kitamura has nothing to disclose.
Naomi Kanazawa Naomi Kanazawa has nothing to disclose.
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.
Kazutoshi Nishiyama, MD, PhD Kazutoshi Nishiyama, MD, PhD has nothing to disclose.