Clinically based score predicting cryptogenic NORSE at the early stage of status epilepticus. Issue 5 (September 2020)

• Record Type: Journal Article
• Authors: Yanagida, Atsuko; Kanazawa, Naomi; Kaneko, Juntaro; Kaneko, Atsushi; Iwase, Ryoko; Suga, Hiroki; Nonoda, Yutaka; Onozawa, Yuya; Kitamura, Eiji; Nishiyama, Kazutoshi; Iizuka, Takahiro
• Is Part Of: Neurology. Volume 7:Issue 5(2020)
• Publication Date: 2020-09
• Subjects: Neuroimmunology -- Periodicals; Neurology -- Periodicals; 616.8
• DOI: 10.1212/NXI.0000000000000849
• Languages: English
• ISSNs: 2332-7812

Abstract

Abstract : Objective: To determine whether a clinically based score predicts cryptogenic new-onset refractory status epilepticus (C-NORSE) at the early stage of status epilepticus (SE) with prominent motor symptoms (SE-M) of unclear etiology. Methods: The score (range 0–6) included 6 clinical features: highly refractoriness to antiseizure drugs, previously healthy individual, presence of prodromal fever, absence of prodromal psychobehavioral or memory alterations, absence of dyskinesias, and symmetric brain MRI abnormalities (the first 2 mandatory). We retrospectively assessed the usefulness of a high scale score (≥5) in predicting C-NORSE in 83 patients with SE-M of unclear etiology, who underwent testing for neuronal surface antibodies (NS-Abs) between January 2007, and December 2019. Results: Thirty-one (37.3%) patients had a high score. Patients with a high score had more frequent prodromal fever (28/31 vs 24/52), mechanical ventilatory support (31/31 vs 36/52), and symmetric MRI abnormalities (26/31 vs 12/52), had less frequent involuntary movements (2/31 vs 30/52), and had absent prodromal psychobehavioral alterations (0/31 vs 27/52), CSF oligoclonal band detection (0/27 vs 11/38), tumor association (0/31 vs 13/52), or NS-Abs (0/31 vs 29/52) than those with a low score (<5). Thirty-three patients (median age, 27 years; 18 [54.5%] female) were finally regarded as C-NORSE. The sensitivity and specificity of a high score for predicting C-NORSE were 93.9% (95% CI 0.87–0.94) and 100% (95% CI 0.95–1.00), respectively. Conclusions: Patients with a high score in the indicated scale are more likely to have C-NORSE, making it a useful diagnostic tool at the early stage of SE-M before antibody test results become available.