Clinical characterization of unknown/cryptogenic status epilepticus suspected as encephalitis: A multicenter cohort study

Shin, Jai Moo; Koo, Yong Seo; Kim, Young Soo; Kim, Dong Wook; Kim, Kwang Ki; Lee, Seo Young; Kim, Hyun Kyung; Moon, Hee Bom; Lim, Joo Hyun; Byun, Jung Ick; Sunwoo, Jun Sang; Moon, Jangsup; Lee, Sang Kun; Jung, Keun Hwa; Park, Kyung‐Il; Chu, Kon; Kim, Jae-Moon; Cho, Yong Won; Jung, Ki Young

doi:10.1016/j.jneuroim.2017.12.004

Cited by 16 publications

(26 citation statements)

References 25 publications

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“…Data on the first acquired CSF, EEG, and MRI studies were collected. The EEG was described according to the 2012 American Clinical Neurophysiology Society's (ACNS) Standardized Critical Care EEG Terminology (27), which we categorized into background slowing activity, sporadic epileptiform discharge, periodic discharge, and electrographic seizures (11). MRI findings including the location and symmetry of signal changes on fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted imaging (DWI) were recorded (10).…”

Section: Methodsmentioning

confidence: 99%

“…Brain inflammation can also cause SE (10, 11), including central nervous system (CNS) infections and autoimmune encephalitis (12). These conditions can be treated and may result in significantly different outcomes (13–15).…”

Section: Introductionmentioning

confidence: 99%

“…Spatola et al were the first to report that patients with an infectious etiology were older in age and had a more severe clinical presentation at first encounter (20). Subsequently, Shin et al found that patients with an autoimmune etiology were younger (11). Herein, we retrospectively reviewed our patients with SE and an inflammatory etiology over a 10.5-year period.…”

Section: Introductionmentioning

confidence: 99%

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The Different Clinical Features Between Autoimmune and Infectious Status Epilepticus

Lin

et al. 2019

Front. Neurol.

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Objective: The prognosis of status epilepticus (SE) is highly related to the underlying etiology. Inflammation of the central nervous system (CNS), including infection and autoimmune encephalitis, is one of the treatable conditions causing SE. The initial presentation of infectious and autoimmune CNS disorders can be quite similar, which may be difficult to differentiate at the beginning. However, treatment for these entities can be quite different. In this study, we aim to identify the differences in clinical features among patients with infectious and autoimmune SE, which could help the clinicians to select initial investigation and ensuing therapies that may improve overall outcomes. Methods: This was a retrospective study that included 501 patients with SE within a period of 10.5-years. Patients with inflammatory etiology were collected and separated into infectious and autoimmune SE. The symptoms at onset, SE semiology, status epilepticus severity score, and END-IT score at admission, treatment for SE, and outcome (modified Rankin Scale) on discharge and last follow-up were recorded. Data on the first cerebrospinal fluid, electroencephalography, and magnetic resonance imaging were also collected. Results: Forty-six (9.2%) of the 501 patients had SE with inflammatory etiology. Twenty-five (5%) patients were autoimmune SE and 21 (4.2%) were infectious SE. Patients with autoimmune SE have younger age and female predominance. As for clinical presentations, psychosis, non-convulsive SE, and super refractory SE were more common in patients with autoimmune SE. Nevertheless, the prognosis showed no difference between the two groups. Conclusion: The different initial clinical presentations and patient characteristics may provide some clues about the underlying etiology of SE. When inflammatory etiology is suspected in patients with SE, younger age, female sex, psychosis, non-convulsive SE, and super refractory SE are clinical features that suggest an autoimmune etiology.

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Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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The Different Clinical Features Between Autoimmune and Infectious Status Epilepticus

Lin

et al. 2019

Front. Neurol.

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“…Probably some members of this group of patients suffer from unrecognized autoimmune encephalitis. For example, an autoimmune or paraneoplastic etiology was found in 13 to 37% of patients with newly occurring, unprovoked, refractory status epilepticus (9,15,16). It can therefore be assumed that a meaningful number of undetected cases exist and that the incidence of autoimmune encephalitis is actually higher than previously thought.…”

Section: Incidence Of Autoimmune Encephalitismentioning

confidence: 99%

Autoantibody-Mediated Encephalitis

Wandinger

Leypoldt²,

Junker³

2018

Deutsches Ärzteblatt International

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A cute or subacute disorders of wakefulness (quantitative impairment of consciousness, ICD-10 R40.-) and qualitative impairment of consciousness (confusion, impaired orientation, amnesia syndromes; ICD-10 R41.-) are frequently occurring causes of hospitalization. In a large neurological emergency department, quantitative disorders of consciousness were the cardinal symptom in every fifth patient seen (reduced vigilance 9%, epileptic seizures 11%) (1). Around 20 to 30% of all hospital inpatients, 50% of elderly patients, and up to 70% of intensive care patients suffer from delirium, i.e., acute deterioration of alertness, organized cognition, memory, attentiveness, and perception (2, e1-e3). Particularly with delirium syndromes the causes are unclear and the treatment principally comprises supportive measures, e.g., management of systemic infections or electrolyte shifts (3). Recently an additional differential diagnosis has been described, namely a group of previously unknown immune-mediated forms of encephalitis with Summary Background: Acute and subacute disturbances of wakefulness and cognitive function are common neurological manifestations in the hospital and in outpatient care. An important element of the differential diagnosis was described only a few years ago: autoimmune encephalitis, a condition whose diagnosis and treatment pose an interdisciplinary challenge. Methods: This review is based on pertinent publications from the years 2005-2017 that were retrieved by a selective search in PubMed, and on the authors' personal experience and case reports. Results: The incidence of autoimmune encephalitis in Germany is estimated at 8-15 cases per million persons per year. In some patients with psychotic manifestations or impaired consciousness of acute or subacute onset, an autoimmune pathogenesis can be demonstrated by the laboratory detection of autoantibodies against neuronal target antigens (e.g., glutamate receptors). Testing of this type should be performed in patients with inflammatory changes in the cerebrospinal fluid or on magnetic resonance imaging (MRI), or those who have had an otherwise unexplained first epileptic seizure or status epilepticus. The cumulative sensitivity of testing for all potentially causative antineuronal antibodies in patients with clinically defined autoimmune encephalitis is estimated at 60-80 %. Figures on cumulative specificity are currently unavailable. Conclusion: The detection of antineuronal antibodies in patients with the corresponding appropriate symptoms implies the diagnosis of autoimmune encephalitis. Observational studies have shown that rapidly initiated immunosuppressive treatment improves these patients' outcomes. Further studies are needed to determine the positive predictive value of antineuronal antibody detection and to develop further treatment options under randomized and controlled conditions.

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“…Initially, anti-epileptic medications (clonazepam, phenobarbital, etc) and steroids, such as dexamethasone, methylprednisolone and prednisolone, are taken into consideration due to beneficial outcomes reported in previous literature for SRSE patients. [10][11][12] Second-line therapy options taken into consideration include immunotherapy (IVIG, PLEX, cyclophosphamide and rituximab). In fact, it is recommended that immunotherapies should be initiated within 72 hours of initial admission.…”

Section: Introductionmentioning

confidence: 99%

NORSE and Super-Refractory Status Epilepticus: A Single-Center Retrospective Chart Review

Rezayee¹

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First, I would like to thank my thesis advisor, Dr. Marissa Kellogg, for being an amazing mentor and for being extremely understanding of the ongoing unexpected difficulties and obstacles. I cannot thank you enough for allowing me, an undergraduate student, to work alongside you in such an advanced and rigorous field, while also constantly encouraging and providing me with more opportunities for exposure in this field. Thank you to Dr. Madeline Nguyen who has worked alongside me throughout the project to analyze the data and allowed me to increase my exposure in Neuro ICU. Also, a big thank you to Chad Murchison for being a great statistician throughout this study.

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Clinical characterization of unknown/cryptogenic status epilepticus suspected as encephalitis: A multicenter cohort study

Cited by 16 publications

References 25 publications

The Different Clinical Features Between Autoimmune and Infectious Status Epilepticus

The Different Clinical Features Between Autoimmune and Infectious Status Epilepticus

Autoantibody-Mediated Encephalitis

NORSE and Super-Refractory Status Epilepticus: A Single-Center Retrospective Chart Review

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