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Seizure Semiology in Antibody-Associated Autoimmune Encephalitis

320 patients were analyzed for this study. Seizures are a frequent and diagnostically relevant symptom of ab + AE. Whereas NMDAR+ patients had few localizing semiological features, semiology in LGI1+ and GAD+ patients pointed toward a predominant temporal seizure onset. FBDS are pathognomonic for LGI1 + AE. Status epilepticus seems to be more frequent in NMDAR + AE.

Seizures are a frequent and important clinical symptom in the early stages of ab + AE with abs against NMDAR, LGI1, and GAD with relevant effect on diagnosis and disease severity. Patients with NMDAR + AE had only few characteristic semiological features according to the more diffuse cerebral affection, but developing seizures is associated with a more severe disease course. By contrast, semiology in LGI1+ and GAD+ patients clearly pointed to a more focal and temporal seizure onset. FBDS are pathognomonic for LGI1+AE. SE seems to be more frequent for NMDAR + AE.

Late-onset seizures and epilepsy: Electroclinical features suggestive of autoimmune etiology

In conclusion, our study shows that, despite sharing some similarities such as seizure type and onset, older patients with AE can be distinguished from those with Late-onset epilepsy of unknown origin (LOEUO) based on their electroclinical features: in particular, high-frequency focal seizures with ictal autonomic manifestations in the elderly should raise the suspicion of an underlying immune-mediated disorder. Our study also highlights the relevant contribution of AEEG, a noninvasive, economic tool, which might reduce the diagnostic delay in LOEUO, and provide useful information to recognize AE.

The impact of childhood epilepsy on academic performance: A population-based matched cohort study

Conclusion: Young people hospitalized with epilepsy have higher risk of not achieving minimum standards for numeracy and reading and not completing high school compared to matched peers. There is a need for effective strategies and interventions (e.g., early seizure control and improved multidisciplinary management and care coordination) to minimize the potential adverse effect of epilepsy on education and its sequelae such as early school leaving, unemployment and poverty in adulthood

Long-term seizure outcomes in patients with autoimmune encephalitis: A prospective observational registry study update

Results: Of 320 AE patients, 75.9% had acute seizures, among whom >90% of patients had their last seizure within 12 months of disease onset. During our follow-up, 21 (9.3%) patients experienced seizure recurrence. Patients with anti-GABAB R encephalitis had a higher cumulative incidence of seizure recurrence than those with anti-NMDAR (log-rank p = .03) or anti-LGI1/Caspr2 encephalitis (log-rank p = .04). Among patients with anti-NMDAR encephalitis, women had a significantly higher cumulative incidence of seizure recurrence than men (log-rank p = .01). Interictal epileptiform discharges (IEDs) or seizures captured on continuous electroencephalogram (EEG) in the acute phase were identified as potential risk factors for seizure recurrence (p = .04, p = .007). Among 163 patients with ≥24 months of follow-up, five (3.1%) showed persistent seizures and required ongoing antiseizure medications despite aggressive immunotherapy.

Significance: Seizure recurrence occurred in a small number of patients and chronic epilepsy occurred in 3.1% of patients during prolonged follow-up. Across all types of AE, risk factors for seizure recurrence were IEDs or seizures captured on EEG in the acute phase; for anti-NMDAR encephalitis, female sex was also a risk factor.

Pilomotor seizures in autoimmune limbic encephalitis: description of two GAD65 antibodies- related cases and literature review

The prevalence of IP in our LE cohort was of 13.3%, higher than expected. According to the literature review, most cases were associated with LGI1 Ab and showed a good response to immunotherapy. With the contribution of our cases, GAD65 emerged as the second most frequently detected Ab, showing a poor outcome. Our findings widen the spectrum of IP-associated Ab, with the respective prognostic implications.

Long-term seizure outcomes in patients with autoimmune encephalitis: A prospective observational registry study update

Just published~
Results: Of 320 AE patients, 75.9% had acute seizures, among whom more than 90% of patients had their last seizure within 12 months of disease onset. During our follow-up, 21 (9.3%) patients experienced seizure recurrence. Patients with anti-GABAB R encephalitis had a higher cumulative incidence of seizure recurrence than those with anti-NMDAR (log-rank P = 0.03) or anti-LGI1/Caspr2 encephalitis (log-rank P = 0.04). Among patients with anti-NMDAR encephalitis, women had a significantly higher cumulative incidence of seizure recurrence than men (log-rank P = 0.01). Interictal epileptiform discharges (IEDs) or seizures captured on continuous EEG in the acute phase were identified as potential risk factors for seizure recurrence (P=0.04; P=0.007). Among 163 patients with ≥ 24 months of follow-up, five (3.1%) showed persistent seizures and required ongoing anti-seizure medications (ASMs) despite aggressive immunotherapy.

Autoimmune encephalitis as an increasingly recognised cause of non-convulsive status epilepticus: A retrospective, multicentre evaluation of patient characteristics and electroencephalography (EEG) results

Status epilepticus (SE) is a severe condition of unrelenting seizures requiring urgent identification and treatment. SE may be unprovoked, occurring in someone with epilepsy, or may be provoked by acute intracranial disease or metabolic derangement. Increasingly encephalitis, particularly autoimmune types, is reported to cause refractory seizures. Whilst convulsive SE is readily identified, non-convulsive SE (NCSE) can be difficult to identify clinically, and electroencephalography (EEG) is required. Therefore, it is critical to identify the key clinical features associated with NCSE on EEG to inform future use of EEG.

Conclusion: There is increasing evidence that previous or concomitant encephalitis, particularly of autoimmune aetiology, is a common cause of NCSE. Therefore, in patients with altered consciousness NCSE should be considered urgently, especially when encephalitis is suspected and subtle motor signs are present.

Multimodal electrophysiological analyses reveal that reduced synaptic excitatory neurotransmission underlies seizures in a model of NMDAR antibody-mediated encephalitis

While neuropsychiatric features are the most common presenting symptom of NMDAR antibody encephalitis, acute symptomatic seizures and encephalopathic electroencephalogram (EEG) changes are essential clinical and investigative features in patients of all ages. Seizures present as part of the disease course in >80% of patients and EEG changes are seen in >95%, more commonly than Magnetic Resonance Imaging (MRI) brain changes, as noted repeatedly in cohort and case studies.

Autoimmune Encephalitis–Related Seizures and Epilepsy: Diagnostic and Therapeutic Approaches

Highly Recommended 
The identification of neural autoantibodies in patients with new-onset seizures of unknown etiology has led to the concept of acute symptomatic seizures secondary to autoimmune encephalitis (ASSAE) and autoimmune-associated epilepsy (AAE). In these patients, antiseizure medication (ASM)-resistant seizures occur as an early and prominent feature. When combined with other symptoms suggestive of immune-mediated seizures, urgent consultation with neurology is recommended. Early treatment with immunotherapy can drastically alter the course of the disease. First-line immunotherapies include intravenous steroids, intravenous immunoglobulin, and PLEX. In the case of insufficient response, cyclophosphamide and rituximab can be considered appropriate second-line treatments. Treatment also includes adjunct ASM therapy and non-CNS tumor resection (if paraneoplastic etiology is suspected) when found on screening.
As we continue to advance our knowledge in AAE and ASSAE, we need to clarify its definition and diagnostic criteria and promote recognition of relevant symptoms (detailed in Table 2) that would lead to early neurology referral and timely diagnosis. The recently developed APE2 and ACES scores have helped build a foundation for diagnosing ASSAE and AAE; however, there remains much ambiguity.
Supplimental video is included at the end of the paper. Downloadable .mp4

Diagnostic challenges in patients with temporal lobe seizures and features of autoimmune limbic encephalitis

Conclusions: In patients with temporal lobe seizures and additional limbic signs, 17% had neuronal Abs affirming ALE diagnosis. Mediotemporal MRI changes were found in all Ab-positive cases and had a positive likelihood ratio of 2.11 (95% confidence interval 1.51-2.95).

Ketogenic Diet Therapy for the Treatment of Post-encephalitic and Autoimmune-Associated Epilepsies

In addition to its potential role in the treatment of acute refractory status epilepticus in patients with PE and AE, ketogenic diet therapies may be feasible and safe in the management of chronic post-encephalitic and autoimmune-associated epilepsy. Patients with prior history of SE might respond better to KDT. Further studies are needed to explore the efficacy of KDT in managing seizures in patients with PE and AE. Moreover, whether the early use of KDT can alter the pathophysiology, prognosis, and outcome of patients with encephalitis warrants further exploration.

Autoimmune encephalitis and epilepsy: evolving definition and clinical spectrum

n this review, we document the clinical and immunologic characteristics of autoimmune encephalitis known to date, with the goal of helping clinicians in differential diagnosis and to provide prompt and effective treatment.

Specific autoimmune encephalitis syndrome

Although clinical manifestations of autoimmune encephalitis can vary significantly, different autoantibodies can be associated with similar phenotypes, certain clinical features suggest a specific type of autoimmune encephalitis, such as facio-brachial dystonic seizures (FBDS) and memory impairment with anti-LGI1 encephalitis; encephalopathy, insomnia, ataxia, peripheral nerve hyperexcitability, and neuropathic pain with anti-Caspr2 encephalitis; refractory SE with anti-GABAA R encephalitis; and myoclonus, tremors, hyperexcitability, and severe diarrhea with anti-DPPX encephalitis (Table 1). Seizures and movement disorders along with psychosis, confusion, or additional behavioral changes are the most relevant initial manifestations in all age groups.

Autoimmune Encephalitis in Late-Onset Seizures: When to Suspect and How to Treat

AE is an important etiology in late-onset seizures, and seizures may be the first symptom of AE. 

The results of our study provide clinicians with additional information verified in a great cohort of 225 patients on the prevalence and outcomes of AE as well as predictors of when to suspect AE in patients with late-onset seizures. Although characteristic signs of inflammation in AE may be lacking especially in elderly patients, the presence of CSF and MRI signs of inflammation, mesial temporal neuropsychological alterations, younger age, a known malignancy and specific semiological features (such as FBDS) should suggest AE. An epileptic seizure may be the first symptom of AE. In short, AB testing in CSF and sera, cerebral MRI, lumbar puncture, and neuropsychological testing for mesial temporal deficits should be part of the diagnostic protocol for AE following late onset seizures.

Adult-onset temporal lobe epilepsy suspicious for autoimmune pathogenesis: Autoantibody prevalence and clinical correlates

Temporal lobe adult-onset seizures (TAOS) related to autoimmunity represent an increasingly recognized disease syndrome within the spectrum of epilepsies. 

Here, we aimed to gain insights into (a) the distribution of ‘neurological’ autoABs (neuroABs, defined as autoABs targeting neuronal surface structures or ‘onconeuronal’ ABs or anti-glutamate
acid decarboxylase 65 (GAD65) autoABs) in a large consecutive TAOS patient cohort, to
characterize (b) clinical profiles of seropositive versus seronegative individuals and to find (c) potential evidence for other autoABs

Clinical Approach to Autoimmune Epilepsy

Considerable progress has been made in the field of autoimmune epilepsy since the discovery of autoimmune encephalitis. The introduction of various immunotherapy options has made some cases of the disease curable.
This review may help in the development of practical treatment approaches for autoimmune epilepsy—the clues for identifying autoimmune epilepsy suggested in this study provide a basic understanding of when to begin empirical immunotherapy. Moreover, a method has been provided for selecting immunotherapy options according to the pathomechanisms of different types of autoimmune epilepsy.

Commentary on "Autoimmune encephalitis and epilepsy: evolving definition and clinical spectrum"

The central nervous system (CNS) is considered an immune-privileged organ, but recent research revealed that it is an active environment that interacts with diverse immune cells in both the disease state and the healthy brain. Immune processes play a vital role in CNS homeostasis, resilience, and brain reserve, and highly sensitive narrow ranges of inflammatory balance seem to be required for health. Crosstalk between the immune and nervous systems has been revealed in a wide spectrum of neurological diseases. Increased levels of proinflammatory cytokines are found in patients with epilepsy, developmental disorders, neurodegenerative diseases, and psychiatric disorders. Therefore, many neurologic and neurodevelopmental disorders in children might be re-evaluated and investigated in terms of immunologic interactions including autoantibodies, cytokines, and microRNAs.

Seizures and risk of epilepsy in anti-NMDAR, anti-LGI1, and anti-GABA B R encephalitis

Conclusion: Our study suggested that immunotherapy delay and IEDs were associated with the development of epilepsy in patients with anti-NMDAR, anti-LGI1, and anti-GABAB R encephalitis. Early diagnosis and treatment were required, and particular consideration should be given to patients with these risk factors.

Rituximab for Autoimmune Encephalitis with Epilepsy

Rituximab is effective for antibody-associated disorders such as lupus, myasthenia gravis, and neuromyelitis optica. Here, we present three patients who were admitted with recalcitrant status epilepticus and demonstrated serum antibodies against NMDAR, LGI1, or VGCC using a cell-based assay. All patients demonstrated complete, long-term epilepsy control and improvement in symptoms with rituximab.​

Acute Symptomatic Seizures Secondary to Autoimmune Encephalitis and Autoimmune-Associated Epilepsy: Conceptual Definitions

Seizures are a well-recognized and often prominent manifestation of autoimmune encephalitic syndromes. Progress in the detection of pathogenic neural autoantibodies has led to increased awareness of autoimmune causes of seizures. Clinical studies of patients with these autoantibodies have improved our understanding of the seizure characteristics, treatments, and seizure prognosis in these disorders.

This paper defines two main diagnostic entities (distinct situations): (a) acute symptomatic seizures secondary to autoimmune encephalitis, and (b) autoimmune-associated epilepsy, the latter of which suggests an enduring predisposition to seizures. Such a distinction is relevant when discussing the pathophysiology (how the disease functions), treatment, prognosis, and social consequences of these disorders.

IVIG Shows Efficacy for Autoimmune Encephalitis and Epilepsy

Intravenous immunoglobulin (IVIG) is an effective acute treatment strategy for reducing seizures in patients with seizures tied to two rare types of antibody-associated encephalitis, LGI1, and CASPR2, according to results from a small, double-blinded randomized, placebo-controlled trial featured as part of the 2020 AAN Science Advances.

Autoimmune seizures and epilepsy

Highly Recommended paper

Most autoimmune encephalitides present with seizures, but the probability of evolving to epilepsy is relatively small. The risk of epilepsy is higher for disorders in which the antigens are intracellular (often T cell-mediated) compared with disorders in which the antigens are on the cell surface (antibody-mediated).

A common feature of all types of autoimmune epileptic seizures is the refractoriness to antiepileptic drugs unless immunotherapy is concurrently used. It is currently unclear whether some antiepileptics are better than others in patients with these disorders.

Autoimmune Epilepsy A newly recognized category of epilepsy caused by or associated with antibodies

The clinical presentation may be complex and testing may need to be repeated when findings are initially negative or if specific antibodies are not found.  Each antibody has its unique clinical findings, cancer association, and response to treatment. Advances in the detection and identification of causative antibodies and other biomarkers will aid in diagnosis and prompt treatment of these patients in the future.

Autoimmune Epilepsy

Autoimmune Epilepsy is a rapidly expanding field. Check out this review article discussing the latest advances, diagnostics and therapeutics.

Seizures and epilepsy of autoimmune origin: A long-term prospective study

Objective: To follow prospectively a group of patients with seizures or epilepsy and suggestive clinical features of autoimmune aetiology and find out how many are finally diagnosed with acute symptomatic seizures (ASS) secondary to autoimmune encephalitis or autoimmune-related epilepsy, and how many develop epilepsy. Notably, all patients with GAD ab 17/17 (100 %) evolved to chronic disease. Four patients (29 %) with ASS secondary to autoimmune encephalitis developed epilepsy.

Significance: ASS secondary to autoimmune encephalitis or autoimmune-related epilepsy will be diagnosed in nearly half of patients who have been suspected of it. The only diagnostic clue is neuronal ab. Patients who have suffered ASS secondary to autoimmune encephalitis may develop epilepsy over time.

Predisposing factors and prognosis of status epilepticus in patients with autoimmune encephalitis

The incidence of SE in patients with AE in western China was 22.03%. EEG and head MRI abnormalities may be susceptibility factors for SE in patients with AE. Glasgow scores <8 when admitted to hospital, abnormal EEG, and seizure duration >30 minutes were risk factors for a poor prognosis in the status of epilepsy (modified Rankin score 3–6).

Use of diffusion-weighted imaging to distinguish seizure-related change from limbic encephalitis

In patients with medial temporal lobe T2-hyperintensity and one of the diffusion restriction patterns described herein, the signal abnormality may be a peri-ictal phenomenon rather than indicative of LE and should prompt investigation for seizure. Even in patients with LE, these patterns should raise concern for seizure.

Electroclinical features of seizures associated with autoimmune encephalitis

Highlights

Autoimmune seizures frequently involve the medial temporal and perisylvian regions.

Electroclinical features are dynamic and evolve over the course of the disease.
Refractory and frequent seizures are seen in the acute phase.
In patients who continue to experience seizures, no latent period is seen.
Electroclinical features involving temporal lobes predict the persistence of seizures.

New approach to autoimmune epilepsy

The prevalence of autoimmune epilepsy isn’t known. Recent research suggests that people with autoimmune disease are almost four times likelier to have epilepsy than are people without autoimmune disorders. “Autoimmune-mediated epilepsy is probably a lot more common than we think,” Dr. Britton says.

In addition to the presence of neural antibodies, clinical features suggestive of autoimmune epilepsy include:

  • Acute to subacute onset, with seizures occurring every three months or less
  • Multiple types of seizures or faciobrachial dystonic seizures
  • Resistance to anti-seizure medication
  • Personal or family history of autoimmunity
  • History of recent or past neoplasia
  • Viral prodrome
  • Evidence of CNS inflammation

Characteristics of Seizure and Antiepileptic Drug

Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis

Neurological Autoantibody Prevalence in Epilepsy of Unknown Etiology

Epilepsy, Autoimmune Evaluation, Spinal Fluid

The hidden epilepsy epidemic with valuable video on all different types of seizures

Pilomotor seizures in autoimmune limbic encephalitis: description of two GAD65 antibodies- related cases and literature review in Chinese patients with epilepsy and encephalopathy scores for patients with different neuronal surface antibodies

Highlights

  • The prevalence of pilomotor seizures in patients affected by limbic encephalitis may be higher than expected.
  • LGI1 and GAD65 emerged as the most frequently associated antibodies to ictal piloerection.
  • SSR recording may help detecting ictal piloerection.

Antibody prevalence and immunotherapy response in Chinese patients with epilepsy and encephalopathy scores for patients with different neuronal surface antibodies

This paper looks from the epileptologist’s perspective: how many patients with epileptic seizures have actually AE antibodies.

Autoimmune-Associated Epilepsy When multiple anticonvulsant and immunotherapy trials fail, what next?

In this case report it presents the case of a patient who initially was diagnosed with limbic encephalitis after presenting with seizures. He had treatment and improved but then seemed to have additional relapse and received additional treatment. Individuals with new-onset refractory status epilepticus (NORSE) once infectious causes have been ruled out should receive early immunotherapy. Autoimmune encephalitis is the most common cause of NORSE and should be considered highly when making the diagnosis. 

Autoimmune encephalitis and seizures, cerebrospinal fluid, imaging, and EEG findings: a case series

This is a retrospective study of 37 adults 18 years or older with autoimmune encephalitis due to antibodies against membrane surface antigens as well as anti-glutamic acid decarboxylase (anti-GAD) antibodies.

Autoimmune epilepsy due to N-methyl-d-aspartate receptor antibodies in a child: a case report

Our case report highlights the importance of early diagnosis of AEp, early treatment with immunotherapy, long-term clinical follow-up, and timely escalation and continuation of immunotherapy in achieving a good patient outcome, retaining intellectual development, and quality of life.

Super-refractory status epilepticus in autoimmune encephalitis treated with interleukin-1 receptor antagonist, anakinra

Autoimmune encephalitis is increasingly recognized as a major cause of new-onset refractory status epilepticus. We propose that the successful treatment with anakinra in our case could be due to elevated inflammatory cytokines in the pathogenesis of autoimmune encephalitis, although we acknowledge that interleukin levels were unfortunately not available. We conclude that anakinra can be a valuable alternative option in patients with autoimmune encephalitis refractory to conventional immunotherapies.

Autoimmune Encephalitis and Epilepsy: Evolving Definition and Clinical Spectrum

This form of encephalitis can involve children with multifaceted presentation of seizures and unexpected behavioral changes. The spectrum of neuropsychiatric symptoms in children is less definitive than in adults, and the incorporation of clinical, immunological, electrophysiological and neuroradiological results is critical for a diagnostic approach. In this review, we document the most appropriate data both clinical and immunologic characteristics of the autoimmune encephalitis known so far, with the goals of assisting clinicians in the differential diagnosis and providing a promptly effective treatment.

Association between autoimmune encephalitis and epilepsy: Systematic review and meta-analysis

Highlights:

Diverse neuronal antibodies,such as anti-NMDAR, anti-AMPAR, anti-GABA-AR, anti-GABA-BR, anti-LGI1, and etc., were found to be associated with autoimmune encephalitis (AE) and AE-related epilepsy.
Epidemiologically, there are still gaps in demonstrating the combination of AE with seizures, the development of AE to epilepsy, and the positive rate of antibodies in patients with epilepsy.
Understanding the epidemiology of AE and AE-related epilepsy is crucial and beneficial for early health care management, therapeutic strategies and decision, as well as prognosis prediction.

Diagnostic utility of cerebrospinal fluid (CSF) findings in seizures and epilepsy with and without autoimmune-associated disease

This review aims to outline different CSF parameters and their alterations in seizures or epilepsy. Some routine CSF parameters are frequently altered after seizures, but are not specific such as CSF protein and lactate. Pleocytosis and CSF specific oligoclonal bands are rare and should be considered as signs of infectious or immune mediated seizures and epilepsy. Markers of neuronal damage show conflicting results, and are as yet not established in clinical practice. Parameters of neuronal degeneration and more specific immune parameters are less well studied, and are areas of further research. CSF analysis in new-onset seizures or status epilepticus serves well in the differential diagnosis of seizure etiology. Here, considerations should include autoimmune-associated seizures. CSF findings in these disorders are a special focus of this review and are summarized in a comprehensive overview. Until now, CSF analysis has not yielded clinically helpful biomarkers for refractory epilepsy or for assessment of neuronal damage which is a subject of further studies.

F-18 Fluorodeoxyglucose Positron Emission Tomography Metabolic Phenotype in Myelin Oligodendrocyte Glycoprotein Antibody-Positive Autoimmune Epilepsy

We describe the metabolic phenotype on F-18 fluorodeoxyglucose positron emission tomography (PET) in a 13-year-old female with myelin oligodendrocyte glycoprotein (MOG) antibody-positive encephalitis. Unilateral hemispheric hypometabolism on PET may be the metabolic phenotype of autoimmune epilepsy associated with MOG antibody.

Super-refractory status epilepticus in autoimmune encephalitis treated with interleukin-1 receptor antagonist, anakinra

T cell numbers correlate with neuronal loss rather than with seizure activity in medial temporal lobe epilepsy

We here provide a detailed histopathological investigation of the involvement of T cells in various subgroups of Medial temporal lobe epilepsy (MTLE), which suggests that T cell influx
correlates to neuronal loss rather than seizure activity.

Acute symptomatic seizures secondary to autoimmune encephalitis and autoimmune‐associated epilepsy: Conceptual definitions

Key Points

  • We support using the term “acute symptomatic seizures secondary to autoimmune encephalitis” to refer to seizures occurring in the setting of the active phase of immune‐mediated encephalitis.
  • We suggest the term “autoimmune‐associated epilepsy” to refer to chronic seizures determined to be secondary to autoimmune brain diseases.
  • Autoimmune‐associated epilepsy may stem from ongoing brain autoimmunity and also from associated structural brain abnormalities.
  • The distinction between acute symptomatic seizures secondary to autoimmune encephalitis and autoimmune‐associated epilepsy has clinical and therapeutic implications.

Characteristics and outcome‐related factors of seizure at the first onset of autoimmune encephalitis: A retrospective study

Seizure outcome of autoimmune encephalitis (AE) varies from seizure‐free to refractory epilepsy, and the associated factors remain unclear. We aimed to describe seizure characteristics, identify seizure outcome‐related factors, and discuss the medication strategy of antiepileptic drugs (AEDs) at the first onset of AE.

5 CONCLUSIONS

In the acute phase of AE, seizure characteristics may be considered in the selection and utilization of AEDs. Onset with seizure and SE occurrence may lead to a poor seizure outcome, while human gamma globulin administration and low antibody titer may contribute to a good seizure outcome. For patients who have achieved seizure‐free status in the acute phase, the factors mentioned above may be considered in the withdrawal strategy of AEDs after the acute phase. Early identification of patients qualified to discontinue AEDs can avoid the additional adverse effects and high costs of AEDs, thereby easing the treatment burden borne by patients.

Long-term seizure outcome and antiseizure medication use in autoimmune encephalitis

Results

Out of 94 AE patients, 75 were analyzed; 47 patients had NMDAR, 17 LGI1, 7 GAD, 3 CASPR2, and 1 mGluR5 antibodies. Fifty-three of the 75 patients (71 %) experienced seizures, all of which for the first time occurred at AE onset. After a median follow-up of 6 years (range, 1–15), 47 of the 53 AE patients had 1-year terminal seizure remission, median duration of terminal seizure freedom was 5 years. Rate of 1-year terminal seizure remission was significantly higher in patients with neuronal surface antibodies (NMDAR 97 %, LGI1 93 %, CASPR2 100 %) compared to patients with GAD antibodies (20 %, p < 0.001). In seizure-free patients, ASM was withdrawn after 13 months (median) without any relapse seizures.

Conclusions

Seizures are common in most forms of AE manifesting at disease onset in all cases. However, the development of AAE is rare and typically occurs in patients with GAD antibodies. Thus, in most AE cases with neuronal surface antibodies, ASM can be withdrawn after the acute phase of AE with low risk of seizure relapse.

Seizures in autoimmune encephalitis-A systematic review and quantitative synthesis

Of 3722 antibody-positive AE patients, 2601 (69.9%) had clinical seizures during the course of their illness. Of the 2025 patients with antibody-positive AE and available EEG data, 1718 (84.8%) had some EEG abnormality (eg, epileptiform discharges, slowing, and so on). Anti- N-methyl-d-aspartate (NMDA) receptor encephalitis (anti-NMDARE) was the most commonly reported type of AE (1985/3722, 53.3%). Of the anti-NMDARE patients with available seizure or EEG data, 71.8% (n = 1425/1985) had clinical seizures during their illness, and 89.7% (n = 1172/1306) had EEG abnormalities. For all AE patients and in the anti-NMDARE subpopulation, seizures were more common in younger patients (p < .05).

Immunologic Treatments of Seizures and Status Epilepticus

The role of autoimmunity in epilepsy has been highlighted in the literature and the International League Against Epilepsy now recognizes autoimmune epilepsy as a distinct entity. An appropriate and thorough work-up of all new-onset seizures and status epilepticus is paramount in determining the likely efficacy of immunotherapeutic agents in treating seizures and status epilepticus. Criteria for the clinical diagnosis of autoimmune mediated epilepsy and encephalitis have been published by expert consensus and validated models to predict response to immunotherapy exist. These guidelines should guide clinicians about when to promptly start immunotherapy. Immunotherapy has been shown to improve outcomes and may reduce relapse rates in autoimmune encephalitis.

What are Faciobrachial Dystonic Seizures? (seen in LGI1 AE)

In faciobrachial dystonic seizures, autoantibodies mistake our brain tissue as foreign and attack it. This results in symptoms of involuntary jerking of an arm and of the face on the same side of the body. Initially, the jerking may not happen very often but this condition is progressive and some people will have more than 100 seizures in a day. These seizures also cause memory loss, which starts out minimal but becomes worse, to the point a person may not be able to be left alone.

Epileptic seizures are very different than faciobrachial dystonic seizures and are caused by abnormal electrical activity within the brain. They can have very different symptoms based on what area of the brain is affected.

It’s easy to remember the symptoms associated with faciobrachial dystonic seizures when you look closer at the word itself. Facio refers to face and brachial refers to arm, which we know are the primary symptoms in this types of seizures.

Unraveling Autoimmune Epilepsy

 Easy Read Article. “Autoimmune epilepsy is essentially a seizure disorder where a neural-specific antibody is present, may be pathogenic, and may cause seizures,” said Lindsay Higdon, MD, a neurologist and epilepsy specialist at Jefferson University Hospital in Philadelphia, Pennsylvania. In one study, experts found that approximately 20% of unexplained epilepsy cases had evidence of circulating neural antibodies, suggesting an autoimmune cause.1 These antibodies can target antigens present on neural surfaces or within the neural cell.

This article provides an overview of the clinical features most commonly associated with positive antibody testing in epilepsy and the scales that are currently available to screen patients for antibody testing and response to immunotherapy.

Autoimmune Epilepsy in Children: Unraveling the Mystery

 Recent developments have expanded the definition of epilepsy-related to autoimmune mechanisms. Based on current knowledge, autoimmune epilepsy can best be thought of as a subset of autoimmune encephalitis where seizures and epilepsy are the primary presenting factor. Autoimmune epilepsy has been increasingly recognized as a contributor to drug-resistant epilepsies; however, identification of affected individuals remains challenging, particularly in the pediatric population.

This article provides an overview of the clinical features most commonly associated with positive antibody testing in epilepsy and the scales that are currently available to screen patients for antibody testing and response to immunotherapy.

Predictors of neural-specific autoantibodies and immunotherapy response in patients with cognitive dysfunction

Recent studies have validated the use of Antibody-Prevalence-in-Epilepsy (APE) and Responsive-to-immunotherapy-in-Epilepsy (RITE) scores in the evaluation and management of autoimmune-epilepsy.

Autoimmune Epilepsy Clinical Characteristics and Response to Immunotherapy

When clinical and serological clues suggest an autoimmune basis for medically intractable epilepsy, early-initiated immunotherapy may improve seizure outcome.

Seizures and movement disorders: phenomenology, diagnostic challenges and therapeutic approaches

A review of the literature to explore the risk of seizures in movement disorders (MDs)

Predictive models in the diagnosis and treatment of autoimmune epilepsy

Antieplieptic drug therapy in patients with autoimmune epilepsy

Epilepsy, Autoimmune Evaluation, Serum

Epilepsies by Etiology antibody mediated

Progressive Brain Atrophy in Super-refractory Status Epilepticus

Antibody-associated epilepsies: Clinical features, evidence for immunotherapies and future research questions

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International Autoimmune Encephalitis Society is a charitable non-profit 501(c)(3) organization founded in 2016 by Tabitha Andrews Orth, Gene Desotell and Anji Hogan-Fesler. Tax ID# 81-3752344. Donations raised directly supports research, patients, families and caregivers impacted by autoimmune encephalitis and to educating healthcare communities around the world. Financial statement will be made available upon request.

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