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Abstract Details

A Clinically-Based Score that predicts Cryptogenic New-Onset Refractory Status Epilepticus (C-NORSE)
Autoimmune Neurology
S11 - Autoimmune CNS Inflammatory Disorders: Clinical Advances (4:03 PM-4:14 PM)
004

NORSE is a condition characterized by refractory SE without readily identifiable cause in otherwise healthy individuals. When the cause remains unknown despite extensive work-up, it is called C-NORSE. To facilitate the prompt recognition of C-NORSE (e.g., before neuronal antibody test results are available) we recently developed a score (range, 0-6) based on 6 clinical features: 1) Refractoriness to anti-epileptic drugs, 2) previously healthy individual, 3) presence of prodromal high fever, 4) absence of prodromal behavioral or memory alterations, 5) absence of dyskinesias, and 6) symmetric brain MRI abnormalities (Neurol Neuroimmunol Neuroinflamm 2017;4[6]:e396). Each of these features represents 1 point, but the first 2 clinical features were mandatory, and a high score (≥5) at early disease stage predicted C-NORSE.

To validate a clinically-based score that predicts cryptogenic new-onset refractory status epilepticus (C-NORSE) at early stage of status epilepticus (SE).

 

The usefulness of the indicated scale score in predicting C-NORSE was assessed in 78 patients who presented with SE of unclear etiology (before neuronal antibody tests results were known).

Patients with a high score (≥5) (n=23, median age 25 years) had more frequent prodromal fever, mechanical ventilation support, and symmetric MRI abnormalities, and had less frequent dyskinesias, and absent prodromal psycho-behavioral symptoms, CSF oligoclonal bands, tumor association or neuronal antibodies than those with a low score (≤4) (n=55, median age 25 years). The 78 patients with SE were finally diagnosed with autoimmune encephalitis with neuronal antibodies (n=30, with NMDAR [n=28], GABAaR [n=1], or unknown [n=1]), C-NORSE (n=24), and other miscellaneous diseases (n=24). The sensitivity and specificity of a high score for predicting C-NORSE were 95.8% (23/24) and 100.0% (54/54), respectively.

Patients with a high score in the indicated scale are more likely to have C-NORSE, making it a useful diagnostic tool at early stage of SE before antibody test results become available.  

 

Authors/Disclosures
Takahiro Iizuka, MD (Department of Neurology, Kitasato University School of Medicine)
PRESENTER
The institution of Dr. Iizuka has received research support from EUROIMMUN Japan Co., Ltd.
Juntaro Kaneko Juntaro Kaneko has nothing to disclose.
Atsushi Kaneko No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file
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.