Clinical and electrographic features of persistent seizures and status epilepticus associated with anti‐NMDA receptor encephalitis (anti‐NMDARE)

Gofshteyn, Jacqueline S.; Yeshokumar, Anusha K.; Thakur, Kiran T.; Luche, Nicole; Yozawitz, Elissa; Varnado, Shelley; Klenofsky, Britany; Tuohy, Mary Claire; Ankam, Jyoti; Torres, Sarah; Hesdorffer, Dale C.; Nelson, Aaron; Wolf, Steven M.; McGoldrick, Patricia; Yan, Helena; Basma, Natasha; Grinspan, Zachary M.

doi:10.1684/epd.2020.1218

Cited by 6 publications

(3 citation statements)

References 36 publications

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“…Diagnosis of autoimmune encephalitis and demyelinating disorders has become increasingly common in the past decade. [23][24][25][26] Autoimmune disorders alone represent an important etiology of SE, and autoimmune or paraneoplastic SE may be particularly susceptible to progression to refractory SE. 27,28 In this situation, prompt immunomodulating therapies are often required to terminate seizures.…”

Section: Discussionmentioning

confidence: 99%

“…In our study, it was revealed that SE patients with symptomatic etiologies were exposed to higher risks of unfavorable functional recovery. Diagnosis of autoimmune encephalitis and demyelinating disorders has become increasingly common in the past decade 23–26 . Autoimmune disorders alone represent an important etiology of SE, and autoimmune or paraneoplastic SE may be particularly susceptible to progression to refractory SE 27,28 .…”

Section: Discussionmentioning

confidence: 99%

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The value of serum albumin concentration in predicting functional outcome of status epilepticus: An observational study

Liao

Zeng

et al. 2023

Epileptic Disorders

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Objective: Status epilepticus (SE) is a common neurological emergency with unsatisfying prognoses, and accurate prediction of functional outcome is beneficial in clinical decision-making. The relationship between serum albumin concentration and outcome of SE patients has yet to be unveiled. Methods: Clinical profiles of SE patients admitted to Xiangya Hospital, CentralSouth University, from April 2017 to November 2020, were analyzed retrospectively. Outcomes of SE patients at discharge were divided into two groups based on the modified Rankin Scale (mRS): favorable outcome (mRS: 0-3) and unfavorable outcome (mRS: 4-6).Results: Fifty-one patients were enrolled. Unfavorable functional outcome at discharge was reported in 60.8% (31/51). Serum albumin concentration at admission and the Encephalitis-NCSE-Diazepam resistance-Image abnormalities-Tracheal intubation (END-IT) score remained independent predictors for functional outcome of SE patients. A lower albumin concentration at admission and higher END-IT score indicated a higher chance of unfavorable outcome for SE patients.The cut-off value of serum albumin to predict unfavorable outcome was 35.2 g/L, with a sensitivity of 67.7% and specificity of 85.0%, and an area under the receiver operating characteristic curve (ROC) of .738 (95% CI: .600-.876, p = .004). The preferable END-IT score with optimal sensitivity (74.2%) and specificity (60%) was 2 and the area under the ROC was .742, with 95% CI of .608-.876 (p = .004).Significance: Serum albumin concentration at admission and the END-IT score are two independent predictive factors for short-term outcome of SE patients, moreover, the serum albumin concentration is not inferior to the END-IT score in indicating functional outcome at discharge.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

The value of serum albumin concentration in predicting functional outcome of status epilepticus: An observational study

Liao

Zeng

et al. 2023

Epileptic Disorders

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“…Among them, EDB has been associated with longer hospitalization, ICU admission, and poor long-term outcomes [ 14 , 55 , 58 ], while GRDA was strongly associated with abnormal movements [ 56 ] and treatment-response to second-line therapies [ 59 ]. In contrast, EEG findings in long-term monitoring during the acute phase of the disease have not been found to predict the persistence of seizures in long-term follow up [ 60 ].…”

Section: Clinical and Paraclinical Features As Markers Of Anti-nmdar Encephalitismentioning

confidence: 99%

Current Status of Biomarkers in Anti-N-Methyl-D-Aspartate Receptor Encephalitis

Ciano-Petersen

Cabezudo‐García

Muñiz‐Castrillo

et al. 2021

IJMS

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The discovery of biomarkers in rare diseases is of paramount importance to allow a better diagnosis, improve predictions of outcomes, and prompt the development of new treatments. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a rare autoimmune disorder associated with the presence of antibodies targeting the GluN1 subunit of the NMDAR. Since it was discovered in 2007, large efforts have been made towards the identification of clinical, paraclinical, and molecular biomarkers to better understand the immune mechanisms that govern the course of the disease as well as to define predictors of treatment response and long-term outcomes. However, most of these biomarkers are still in an exploratory phase, with only a few candidates reaching the final phases of the always-complex process of biomarker development, mainly due to the low incidence of the disease and its recent description. Clinical and paraclinical markers are probably the most widely explored in anti-NMDAR encephalitis, five of them combined in a clinical score to predict 1 year outcome. On the contrary, soluble molecules, such as persistent antibody positivity, antibody titers, cytokines, and other inflammatory mediators, have been proposed as biomarkers of clinical activity, inflammation, prognosis, and treatment response, but further studies are required for their clinical validation including larger and more homogenous cohorts of patients. Similarly, genetic susceptibility biomarkers are still in the exploratory phase and, therefore, weak conclusions can for now only be achieved. Thus, further studies are warranted to define biomarkers and unravel the underlying mechanisms driving rare diseases such as anti-NMDAR encephalitis. Future international collaborative studies with prospective designs that enable the enrollment of large cohorts will allow for the identification and validation of novel biomarkers for clinical decision-making.

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Autoimmune‐associated seizure disorders

Smith,

Budhram,

Geis

et al. 2024

Epileptic Disorders

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With the discovery of an expanding number of neural autoantibodies, autoimmune etiologies of seizures have been increasingly recognized. Clinical phenotypes have been identified in association with specific underlying antibodies, allowing an earlier diagnosis. These phenotypes include faciobrachial dystonic seizures with LGI1 encephalitis, neuropsychiatric presentations associated with movement disorders and seizures in NMDA‐receptor encephalitis, and chronic temporal lobe epilepsy in GAD65 neurologic autoimmunity. Prompt recognition of these disorders is important, as some of them are highly responsive to immunotherapy. The response to immunotherapy is highest in patients with encephalitis secondary to antibodies targeting cell surface synaptic antigens. However, the response is less effective in conditions involving antibodies binding intracellular antigens or in Rasmussen syndrome, which are predominantly mediated by cytotoxic T‐cell processes that are associated with irreversible cellular destruction. Autoimmune encephalitides also may have a paraneoplastic etiology, further emphasizing the importance of recognizing these disorders. Finally, autoimmune processes and responses to novel immunotherapies have been reported in new‐onset refractory status epilepticus (NORSE) and febrile infection‐related epilepsy syndrome (FIRES), warranting their inclusion in any current review of autoimmune‐associated seizure disorders.

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Clinical and electrographic features of persistent seizures and status epilepticus associated with anti‐NMDA receptor encephalitis (anti‐NMDARE)

Cited by 6 publications

References 36 publications

The value of serum albumin concentration in predicting functional outcome of status epilepticus: An observational study

The value of serum albumin concentration in predicting functional outcome of status epilepticus: An observational study

Current Status of Biomarkers in Anti-N-Methyl-D-Aspartate Receptor Encephalitis

Autoimmune‐associated seizure disorders

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