scholarly journals Clinically based score predicting cryptogenic NORSE at the early stage of status epilepticus

2020 ◽  
Vol 7 (5) ◽  
pp. e849
Author(s):  
Atsuko Yanagida ◽  
Naomi Kanazawa ◽  
Juntaro Kaneko ◽  
Atsushi Kaneko ◽  
Ryoko Iwase ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Early Stage ◽  
Brain Mri ◽  
Ventilatory Support ◽  
Antibody Test ◽  
Refractory Status Epilepticus ◽  
Test Results ◽  
Tumor Association ◽  
Unclear Etiology ◽  
Score Range

ObjectiveTo 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.MethodsThe 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.ResultsThirty-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.ConclusionsPatients 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.

scholarly journals Cryptogenic NORSE

2017 ◽  
Vol 4 (6) ◽  
pp. e396 ◽  
Author(s):  
Takahiro Iizuka ◽  
Naomi Kanazawa ◽  
Juntaro Kaneko ◽  
Naomi Tominaga ◽  
Yutaka Nonoda ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Clinical Features ◽  
Brain Mri ◽  
Ventilatory Support ◽  
Autoimmune Encephalitis ◽  
Refractory Status Epilepticus ◽  
Oligoclonal Bands ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Nmda Receptor Encephalitis

Objective:To report the distinctive clinical features of cryptogenic new-onset refractory status epilepticus (C-NORSE) and the C-NORSE score based on initial clinical assessments.Methods:A retrospective study was conducted for 136 patients with clinically suspected autoimmune encephalitis who underwent testing for autoantibodies to neuronal surface antigens between January 1, 2007, and August 31, 2016. Eleven patients with C-NORSE were identified. Their clinical features were compared with those of 32 patients with anti-NMDA receptor encephalitis (NMDARE).Results:The clinical outcome of 11 patients (median age, 27 years; 7 [64%] women) with C-NORSE was evaluated after a median follow-up of 11 months (range, 6–111 months). Status epilepticus was frequently preceded by fever (10/11 [91%]). Brain MRIs showed symmetric T2/fluid-attenuated inversion recovery hyperintensities (8/11 [73%]) and brain atrophy (9/11 [82%]). Only 2 of the 10 treated patients responded to the first-line immunotherapy, and 4 of the 5 patients treated with IV cyclophosphamide responded to the therapy. The long-term outcome was poor in 8 patients (73%). Compared with 32 patients with NMDARE (median age, 27 years; 24 [75%] women), those with C-NORSE had more frequent prodromal fever, status epilepticus, ventilatory support, and symmetric brain MRI abnormalities, had less frequent involuntary movements, absent psychobehavioral symptoms, CSF oligoclonal bands, or tumor association, and had a worse outcome. The C-NORSE score was higher in patients with C-NORSE than those with NMDARE.Conclusions:Patients with C-NORSE have a spectrum of clinical-immunological features different from those with NMDARE. The C-NORSE score may be useful for discrimination between them. Some patients could respond to immunotherapy.

scholarly journals China NMPA perspective on clinical evaluation of SARS-CoV-2 antibody test reagents in the process of emergency approval

Bioanalysis ◽  
2020 ◽  
Author(s):  
Yunfeng Lv ◽  
Jingyun He ◽  
Rongzhi Liu ◽  
Yu Gao ◽  
Chao Xu ◽  
...  
Keyword(s):  
Early Stage ◽  
Antibody Test ◽  
Standard Test ◽  
Test Method ◽  
Antibody Detection ◽  
Test Results ◽  
Antibody Testing ◽  
National Medical ◽  
Key Points ◽  
Antigen Antibody

Coronavirus disease 2019 (COVID-19) pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). The SARS-CoV-2 antibody testing an important supplement to nucleic acid testing. In the process of emergency approval, the Center for Medical Device Evaluation of the China National Medical Products Administration released The Key Points of Technical Review for the Registration of SARS-CoV-2 Antigen/Antibody Detection Reagents. The Clinical Study Requirement section of the Key Point has put forward requirements in terms of reference methods and subject enrolment among others, which can ensure that the test results can meet the clinical needs. This article draws on the experience of the China NMPA in evaluating diagnostic reagents used to supplement the gold standard test method in the early stage of an epidemic of an infectious disease, as well as to serve as reference for clinicians and regulators.

scholarly journals Status Epilepticus and Neurosyphilis: A Case Report and a Narrative Review

NeuroSci ◽  
2021 ◽  
Vol 2 (4) ◽  
pp. 416-426
Author(s):  
Giada Giovannini ◽  
Stefano Meletti
Keyword(s):  
Status Epilepticus ◽  
Limbic Encephalitis ◽  
Brain Mri ◽  
Refractory Status Epilepticus ◽  
Unknown Etiology ◽  
Neurologic Sequelae ◽  
Life Threatening ◽  
Temporal Structures ◽  
History Of ◽  
New Onset

Neurosyphilis is a rare but life-threatening complication of syphilis that can develop even decades after the primary infection and can be unrecognized. Seizures and status epilepticus (SE) may represent the first manifestation in a previously undiagnosed syphilitic patient. We present an exemplification case of a new onset refractory status epilepticus caused by neurosyphilis and we reviewed the existing literature. We selected all studies reporting cases of SE in the context both of patients with a known diagnosis of syphilis and as the first manifestation of neurosyphilis. We identified 50 patients, mostly composed of immunocompetent, middle-aged males. Thirty-nine patients (83%) presented a new onset SE. A history of subtle and rapidly progressive mood and/or cognitive impairment suggesting a limbic encephalitis-like presentation was frequently observed. Focal frontal or temporal SE was reported in 26. Brain MRI frequently showed T2/FLAIR hyperintensities widely involving the medial temporal structures and the frontal lobes. This review should increase the clinician’s awareness of neurosyphilis as a possible etiology of a new onset SE of unknown etiology, especially in the context of a “limbic encephalitis”-like clinical presentation. Prompt recognition and treatment for neurosyphilis partially or completely reverse neurologic sequelae, changing the natural history of the disease.

How to interpret borderline HCV antibody test results? A comparative study investigating four different anti-HCV assays

2013 ◽  
Vol 51 (01) ◽  
Author(s):  
B Maasoumy ◽  
B Bremer ◽  
R Raupach ◽  
P Lehmann ◽  
MP Manns ◽  
...  
Keyword(s):  
Comparative Study ◽  
Antibody Test ◽  
Test Results ◽  
Hcv Antibody

scholarly journals How to Help Your Patients Enroll in the New-Onset Refractory Status Epilepticus (NORSE) and Febrile Infection-Related Epilepsy Syndrome (FIRES) Family Registry, and Other Rare Epilepsy Registries

2021 ◽  
pp. 153575972199832
Author(s):  
Karnig Kazazian ◽  
Marissa Kellogg ◽  
Nora Wong ◽  
Krista Eschbach ◽  
Raquel Farias Moeller ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Refractory Status Epilepticus ◽  
Epilepsy Syndrome ◽  
Neurologic Disorder ◽  
Mortality And Morbidity ◽  
Refractory Status ◽  
Rigorous Research ◽  
Active Epilepsy ◽  
New Onset

New-onset refractory status epilepticus (NORSE) is a rare clinical presentation of refractory status epilepticus (RSE) that occurs in people without active epilepsy or preexisting neurologic disorder. Febrile infection-related epilepsy syndrome (FIRES) is a subcategory of NORSE. New-onset refractory status epilepticus/FIRES are becoming increasingly recognized; however, information pertaining to disease course, clinical outcomes, and survivorship remains limited, and mortality and morbidity are variable, but often high. The objective of the NORSE/FIRES Family Registry is to (1) provide an easily accessible and internationally available multilingual registry into which survivors or NORSE/FIRES surrogates or family members of people affected by NORSE/FIRES or their physicians can enter data in a systematic and rigorous research study from anywhere in the world where internet is available; and (2) to examine past medical history, outcomes, and quality of life for people affected by NORSE/FIRES.

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