Persistent Intrathecal Antibody Synthesis 15 Years After Recovering From Anti– N-methyl-D-aspartate Receptor Encephalitis

Hansen, Hans-Christian; Klingbeil, Christine; Dalmau, Josep; Li, Wenhan; Weißbrich, Benedikt; Wandinger, Klaus‐Peter

doi:10.1001/archneurol.2013.585

Cited by 7 publications

(7 citation statements)

References 6 publications

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“…The disease specificity and clinical relevance of the antibodies in some of the studies are unclear given that the same antibody test-ing resulted in detection of NMDA receptor antibodies in 23% of patients with a wide range of diseases including some that are not immune mediated (346), leading to inaccurate diagnosis and unnecessary immunotherapy (17). Experience by us and others with large series of patients with anti-NMDA receptor encephalitis show that none of these patients developed schizophrenia during the subsequent years of follow-up even though after recovery some patients had circulating low titers of NMDA receptor antibodies (120,128,309,326).…”

Section: Other Nmda Receptor Antibodies In Other Cns Disordersmentioning

confidence: 99%

Autoantibodies to Synaptic Receptors and Neuronal Cell Surface Proteins in Autoimmune Diseases of the Central Nervous System

Dalmau

Geis

Graus

2017

Physiological Reviews

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504

544

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Investigations in the last 10 years have revealed a new category of neurological diseases mediated by antibodies against cell surface and synaptic proteins. There are currently 16 such diseases all characterized by autoantibodies against neuronal proteins involved in synaptic signaling and plasticity. In clinical practice these findings have changed the diagnostic and treatment approach to potentially lethal, but now treatable, neurological and psychiatric syndromes previously considered idiopathic or not even suspected to be immune-mediated. Studies show that patients' antibodies can impair the surface dynamics of the target receptors eliminating them from synapses (e.g., NMDA receptor), block the function of the antigens without changing their synaptic density (e.g., GABAb receptor), interfere with synaptic protein-protein interactions (LGI1, Caspr2), alter synapse formation (e.g., neurexin-3α), or by unclear mechanisms associate to a new form of tauopathy (IgLON5). Here we first trace the process of discovery of these diseases, describing the triggers and symptoms related to each autoantigen, and then review in detail the structural and functional alterations caused by the autoantibodies with special emphasis in those (NMDA receptor, amphiphysin) that have been modeled in animals.

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Section: Other Nmda Receptor Antibodies In Other Cns Disordersmentioning

confidence: 99%

Autoantibodies to Synaptic Receptors and Neuronal Cell Surface Proteins in Autoimmune Diseases of the Central Nervous System

Dalmau

Geis

Graus

2017

Physiological Reviews

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504

544

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“…Relapse means the occurrence of new symptoms or the aggravation of original symptoms after more than 2 months of the convalescence period. Patients with undetected or recurrent tumors (22) or non-paraneoplastic cases as well as those did not receive timely immunotherapy were more likely to relapse (64). Clinical recovery does not mean the disappearance of serum and CSF antibodies, some patients could still show positive antibodies in serum and CSF (16,65,66).…”

Section: Clinical Implications For Antibody Testingmentioning

confidence: 99%

Autoantibodies detection in anti-N-methyl-D-aspartate receptor encephalitis

Li¹,

Wang²

2023

Ann Transl Med

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Autoimmune encephalitis (AE) covers a group of neurological diseases caused by autoantibodies.AE is severe but treatable. It has attracted more and more attention currently. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most common AE characterized by specific autoantibody mainly against NMDAR subunit 1. Cell-based assays (CBA) on human embryonic kidney 293 (HEK293) cells and immunohistochemistry (IHC) on rat brain tissue have been widely used to detect antibody in patients with AE. However, few studies focused on the overview of these assays detecting autoantibodies in AE. Here we reviewed the detection assays in AE and compared the sensitivity and specificity of CBA and IHC. It's found that IHC got a higher positive rate than CBA in both serum and CSF when screening potential AE, while CBA was more specific. Besides, more positive samples were found in CSF than in serum by either IHC or CBA. Hence, both serum and CSF should be sent to detect antibodies by two assays to avoid misdiagnosis. CSF antibody titers were believed more clinically relevant. When positive results were shown in IHC but negative in CBA, other kinds of antibodies associated AE instead of anti-NMDAR encephalitis should be taken into account. Further studies should pay attention to serum testing for diagnosis or assessment of the disease, as CSF testing is invasive and not always available.

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“…ASM, antiseizure medication; DDD, defined daily dose according to the World Health Organization long time after AIE has completely subsided, especially CASPR2 and NMDAR antibodies. 3,40,50,52 Since there is no generally applicable or universally accepted definition of a "significant change of a titer" or a "low titer," we suggest that the titer decreases as indicated in the table based on published experience. 3,50,52,53 In cases of doubt, the decision of whether an antibody decrease is substantial must be referred to the respective laboratory and its experience of what to consider a relevant reduction or "negative" titer.…”

Section: Criterion 4: Normal Cerebrospinal Fluid Cell Countmentioning

confidence: 99%

What is autoimmune encephalitis‐associated epilepsy? Proposal of a practical definition

Rada

Bien

2023

Epilepsia

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Seizures resulting from cerebral autoimmunity are either acutely symptomatic in the context of autoimmune encephalitis (AIE) with neural surface antibodies, or they are indicative of an enduring predisposition to seizures, that is, epilepsy. Here, we propose a practical definition for autoimmune encephalitis‐associated epilepsy (AEAE): Seizures associated with antibodies against glutamic acid decarboxylase, paraneoplastic syndromes, or Rasmussen encephalitis are classified as AEAE. AEAE secondary to AIE with antibodies against the N‐methyl‐D‐aspartate receptor, leucine‐rich glioma inactivated protein 1, contactin‐associated protein‐2, or γ‐aminobutyric acid‐B receptor can be diagnosed if the following criteria are met: seizures persist for at least 2 years after immunotherapy initiation; no signs of encephalitis on magnetic resonance imaging and no fluorodeoxyglucose positron emission tomography hypermetabolism; normal cerebrospinal fluid cell count; and a substantial decrease in antibody titers. This classification corresponds to different disease mechanisms. While AIE results from the pathogenic effects of neural antibodies, AEAE is probably the consequence of encephalitis‐related tissue damage and thereby mainly structurally mediated. The distinction between AIE and AEAE also has practical consequences: In AIE, immunotherapy is usually highly beneficial, whereas anti‐seizure medication has little effect. In AEAE, immunotherapy is less promising and the usual anti‐seizure interventions are preferable. In addition, the diagnosis of AEAE has social consequences in terms of driving and professional limitations.

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Persistent Intrathecal Antibody Synthesis 15 Years After Recovering From Anti– N-methyl-D-aspartate Receptor Encephalitis

Cited by 7 publications

References 6 publications

Autoantibodies to Synaptic Receptors and Neuronal Cell Surface Proteins in Autoimmune Diseases of the Central Nervous System

Autoantibodies to Synaptic Receptors and Neuronal Cell Surface Proteins in Autoimmune Diseases of the Central Nervous System

Autoantibodies detection in anti-N-methyl-D-aspartate receptor encephalitis

What is autoimmune encephalitis‐associated epilepsy? Proposal of a practical definition

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