Limbic encephalitis as a precipitating event in adult-onset temporal lobe epilepsy

Bien, Christian G.; Urbach, Horst; Schramm, Johannes; Soeder, Bettina M.; Becker, Albert J.; Voltz, Raymond; Vincent, Angela; Elger, Christian E.

doi:10.1212/01.wnl.0000276946.08412.ef

Cited by 217 publications

(178 citation statements)

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“…[2][3][4][5][6][7][8][9] Accumulating evidence supports an autoimmune basis for seizures in the absence of syndromic manifestations of limbic encephalitis for a subset of AED-resistant epilepsy. [10][11][12][13][14][15] Expedited diagnosis is imperative because early initiation of immunotherapy facilitates improvement.…”

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confidence: 99%

Utility of an immunotherapy trial in evaluating patients with presumed autoimmune epilepsy

et al. 2014

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Objective: To evaluate a trial of immunotherapy as an aid to diagnosis in suspected autoimmune epilepsy. Method:We reviewed the charts of 110 patients seen at our autoimmune neurology clinic with seizures as a chief complaint. Twenty-nine patients met the following inclusion criteria: (1) autoimmune epilepsy suspected based on the presence of $1 neural autoantibody (n 5 23), personal or family history or physical stigmata of autoimmunity, and frequent or medically intractable seizures; and (2) initiated a 6-to 12-week trial of IV methylprednisolone (IVMP), IV immune globulin (IVIg), or both. Patients were defined as responders if there was a 50% or greater reduction in seizure frequency.Results: Eighteen patients (62%) responded, of whom 10 (34%) became seizure-free; 52% improved with the first agent. Of those receiving a second agent after not responding to the first, 43% improved. A favorable response correlated with shorter interval between symptom onset and treatment initiation (median 9.5 vs 22 months; p 5 0.048). Responders included 14/16 (87.5%) patients with antibodies to plasma membrane antigens, 2/6 (33%) patients seropositive for glutamic acid decarboxylase 65 antibodies, and 2/6 (33%) patients without detectable antibodies. Of 13 responders followed for more than 6 months after initiating long-term oral immunosuppression, response was sustained in 11 (85%).Conclusions: These retrospective findings justify consideration of a trial of immunotherapy in patients with suspected autoimmune epilepsy. Classification of evidence:This study provides Class IV evidence that in patients with suspected autoimmune epilepsy, IVMP, IVIg, or both improve seizure control. Neurology ® 2014;82:1578-1586 GLOSSARY AED 5 antiepileptic drug; CASPR2 5 contactin-associated protein-like 2; CC 5 calcium channel; gAChR 5 neuronal acetylcholine receptor, ganglionic-type; GAD65 5 glutamic acid decarboxylase 65; IgG 5 immunoglobulin G; IVIg 5 IV immune globulin; IVMP 5 IV methylprednisolone; LGI1 5 leucine-rich, glioma-inactivated 1; PMA Abs 5 antibodies to neural plasma membrane antigen; VGKC 5 voltage-gated potassium channel.Approximately one-third of epilepsy cases are intractable to antiepileptic drug (AED) therapy.

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confidence: 99%

Utility of an immunotherapy trial in evaluating patients with presumed autoimmune epilepsy

et al. 2014

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“…There can be movement disorders with oro-lingual-facial dyskinesia, autonomic manifestations, central hypoventilation, tachycardia and bradycardia [91] [97]. Autoantibodies targeting the NMDA receptor occur with herpes simplex virus encephalitis (HSVE) [98].…”

Section: Anti-nmda Receptor Encephalitismentioning

confidence: 99%

Autoimmune Encephalitides

Younger¹

2017

WJNS

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Autoimmune encephalitis is a potentially severe disorder of the brain of diverse causes with a complex differential diagnosis. Recent advances in the past decade have led to the elucidation of new syndromes and biological markers transforming the approach to diagnosis and management of autoimmune encephalitis. Limbic encephalitis, the commonest form of autoimmune encephalitis, combines common presentations of cognitive, psychiatric, and epileptic disorders and has until recently been considered paraneoplastic or postinfectious in origin. The autoimmune encephalitides are clinically and histopathologically associated with serum and intrathecal antibodies to intracellular and surface neuronal antigens, and constituents of the limbic system neuropil. This has led to a reconsideration of a number of neuropsychiatric and neurocognitive disorders as having shared mechanisms of origin. This chapter reviews their historical background, clinical presentation, laboratory evaluation, histopathology, diagnosis and management. KeywordsAutoimmune, Encephalitis, Hashimoto, Encephalopathy Historical PerspectiveCorsellis and colleagues [1] coined the term limbic encephalitis (LE) in 1968, noting a relation to bronchial cancer in three patients in the sixth to eighth decade of life, and showing close clinicopathologic similarity to cases described by Brierley and colleagues [2] six years earlier. All three cases had subacute temporal lobe seizures, neuropsychiatric, and memory disturbances for two years before death. Postmortem examination showed inflammatory lesions in limbic grey matter sections of the brain, especially in medial temporal lobe structures of the uncus and amygdala nuclei, and hippocampal, cingulum and dentate gyri. Case 2 had an undifferentiated non-metastatic lung carcinoma removed six months after onset of neurological symptoms, while two others had a clinically unsus- The formulation of the relation of GAD antibodies to SPS has been especially instructive in understanding how far the science of autoimmune neurologic disorder has advanced. In 1988, Solimena and colleagues [28] investigated the existence of non-paraneoplastic CNS autoimmunity in a patient with SPS, epilepsy and type-1 diabetes (T1D), and increased titers of oligoclonal CSF IgG. Both the serum and CSF produced identical intense staining of all gray-matter regions. GAD65 was thence an important autoantigen in T1D, being highly expressed in the cytoplasm of pancreatic β cells. GAD-derived peptides were presentable by main histocompatibility complex (MHC) class I molecules and recognized by CD8+ cells on the surface of β cells [29]. Activation of CD8+ GAD-specific T-cells further differentiated into memory cells suggesting a pathogenic role in T1D [30]. However, only patients with very high titers of GAD were associated with LE [31], and they typically presented with recent-onset temporal lobe epilepsy (TLE) and intrathecal secretion, defining a form of non-paraneoplastic LE. Other patients within the SPS spectrum harbored antibodies agains...

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“…[31] Erişkin başlangıçlı hipokampal sklerozlu temporal lob epilepsisi tanılı hastalarda geçirilmiş limbik ensefalit öyküsü bildirilmiş olup, voltaj bağımlı potasyum kanalı kompleksine (VGKCc) karşı gelişmiş antikorların saptandığı limbik ensefalit tablosunda da iki yanlı meziyal temporal skleroz geliştiği gösterilmiştir. [32,33] Öte yandan tanımlanan bu antikorların subklinik seyretmiş bir ensefalit tablosunun bir göstergesi olup olmadığı ve patogenezdeki rolleri tam olarak aydınlatılamamıştır. Bu antikorların tesadüfi eşlikçiler olabilecekleri üzerinde de durulmaktadır.…”

Section: Tedavi Yaklaşımlarıunclassified

Immunotherapy Responsive Epilepsies: General Clinical Features, Commonly Detected Antibodies and Treatment Studies

Eki̇zoğlu¹

2015

Epilepsi

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SummaryThe role of immunity and inflammation in epilepsy has long been considered and in recent years, discovery of some antibodies against to various neuronal antigens especially in cases refractory to conventional anti-epileptic drugs raised the concept of "autoimmune epilepsy". Immunotherapy may lead to significant benefits in cases with suspicion of autoimmune etiology. In this paper, epileptic pictures with a known autoimmune aspect as well as those pictures with recent increased awareness due to the discovery of neuronal auto-antibodies responding to immunotherapies and their management options are summarized.Keywords: Epilepsy; immunotherapy; autoantibodies; autoimmunity. ÖzetEpilepside immünite ve enflamasyonun rolü üzerinde uzun zamandır durulmakta olup, son yıllarda özellikle geleneksel antiepileptik ilaçlara dirençli olgularda birtakım nöronal antijenlere karşı gelişen antikorların saptanması ile "otoimmün epilepsi" kavramı doğmuştur. Otoimmün etiyolojiden şüphelenilen olgularda immün tedavinin başarılı olduğu görülmüştür. Bu yazıda otoimmün boyutu bilinen epilepsi tablolarının yanısıra, son yıllarda önemi artan immüntedaviye yanıtlı nöronal otoantikorlarla ilişkili tablolar ve tedavi seçenekleri özetlenmiştir.

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Limbic encephalitis as a precipitating event in adult-onset temporal lobe epilepsy

Abstract: Temporal lobe epilepsy with hippocampal sclerosis can manifest in adult life. Around half the patients have evidence consistent with an autoimmune process. If confirmed, this should have implications for diagnosis, prevention, and treatment.

Cited by 217 publications

References 37 publications

Utility of an immunotherapy trial in evaluating patients with presumed autoimmune epilepsy

Utility of an immunotherapy trial in evaluating patients with presumed autoimmune epilepsy

Autoimmune Encephalitides

Immunotherapy Responsive Epilepsies: General Clinical Features, Commonly Detected Antibodies and Treatment Studies

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