Characterization of a Subtype of Autoimmune Encephalitis With Anti–Contactin-Associated Protein-like 2 Antibodies in the Cerebrospinal Fluid, Prominent Limbic Symptoms, and Seizures

Joubert, Bastien; Saint‐Martin, Margaux; Noraz, Nelly; Picard, Géraldine; Rogemond, Véronique; Ducray, François; Desestret, Virginie; Psimaras, Dimitri; Delattre, Jean‐Yves; Antoine, Jean‐Christophe; Honnorat, Jérôme

doi:10.1001/jamaneurol.2016.1585

Cited by 161 publications

(147 citation statements)

References 19 publications

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“…In contrast, there may be prolonged disease periods with "seizures only" with LGI1 antibodies (18% [25]). One or more "seizure(s) only" as a presenting symptom are frequent with CASPR2 antibodies (24% [26] to 72% [27]) and in male patients with anti-NMDAR LGI1, CASPR2. NMDAR, GlyR, AMPAR, GABA B R, mGluR5, DPPX, GABA A R, Neurexin-3α, GAD, Hu, Ma, CV2, amphiphysin, DNER, Sox1…”

Section: What Does a "Positive Result" Mean?mentioning

confidence: 99%

“Autoimmune Epilepsy”: Encephalitis with Autoantibodies for Epileptologists

Bien

Holtkamp

2017

Epilepsy Curr

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Autoimmune encephalitides may account for epilepsies of so far unknown cause. These “autoimmune epilepsies” may respond well to immunotherapy. More than a dozen autoantibodies have been found with this constellation; therefore, broad autoantibody testing of serum-CSF pairs offers the best diagnostic yield. Several particular features raise the suspicion of an autoimmune cause in otherwise unexplained seizure disorders.

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Section: What Does a "Positive Result" Mean?mentioning

confidence: 99%

“Autoimmune Epilepsy”: Encephalitis with Autoantibodies for Epileptologists

Bien

Holtkamp

2017

Epilepsy Curr

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“…They compared patients with anti-Caspr2 antibodies in the CSF to patients presenting neuromyotonia or Morvan’s syndrome with anti-Caspr2 antibodies in the serum only. In this work, they showed that the presence of anti-Capsr2 antibodies in the CSF is associated with a much more homogeneous clinical pattern of autoimmune encephalitis, characterized by a prevalent limbic involvement and seizure occurrence 59. These results emphasize the lack of functional studies focusing on antibody specificities and the importance of testing both serum and CSF samples.…”

Section: Othersmentioning

confidence: 69%

“…Identification of these antigens helped to clarify the apparent diversity of symptoms attributed to voltage-gated potassium channels antibodies. While anti-Lgi1 antibodies are preferentially associated with classical limbic encephalitis, anti-Capsr2 antibodies are associated with Morvan’s syndrome, neuromyotonia, and sometimes with neuropathies or limbic encephalitis 52,58,59…”

Section: Anti-lgi1 Encephalitismentioning

confidence: 99%

“…Detection of Capsr2 antibodies in patients with neuromyotonia is often correlated with an underlying tumor (frequently a thymoma),66 and patients having a tumor associated with anti-Caspr2 have a poorer prognosis. A recent work of Joubert et al59 reveals that the apparent clinical diversity of anti-Caspr2 encephalitis may be linked to the site of antibody synthesis. They compared patients with anti-Caspr2 antibodies in the CSF to patients presenting neuromyotonia or Morvan’s syndrome with anti-Caspr2 antibodies in the serum only.…”

Section: Othersmentioning

confidence: 99%

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Autoimmune encephalitis in psychiatric institutions: current perspectives

Bost¹,

Pascual²,

Honnorat³

2016

NDT

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Autoimmune encephalitis is a rare and newly described group of diseases involving autoantibodies directed against synaptic and neuronal cell surface antigens. It comprises a wide range of neuropsychiatric symptoms. Sensitive and specific diagnostic tests such as cell-based assay are primordial for the detection of neuronal cell surface antibodies in patients’ cerebrospinal fluid or serum and determine the treatment and follow-up of the patients. As neurological symptoms are fairly well described in the literature, this review focuses on the nature of psychiatric symptoms occurring at the onset or during the course of the diseases. In order to help the diagnosis, the main neurological symptoms of the most representative synaptic and neuronal cell surface autoantibodies were detailed. Finally, the exploration of these autoantibodies for almost a decade allowed us to present an overview of autoimmune encephalitis incidence in psychiatric disease and the general guidelines for the management of psychiatric manifestations. For the majority of autoimmune encephalitis, the prognosis depends on the rapidity of the detection, identification, and the management of the disease. Because the presence of pronounced psychiatric symptoms drives patients to psychiatric institutions and can hinder the diagnosis, the aim of this work is to provide clues to help earlier detection by physicians and thus provide better medical care to patients.

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“…CASPR2 is a transmembrane protein found in the juxtaparanodes of myelinated axons and can be complexed with voltage-gated potassium channels to maintain electrical functioning of neurons 9. Anti-CASPR2 antibodies in CSF have been associated with rare cases of autoimmune (non-paraneoplastic) limbic encephalitis 10 11…”

Section: Discussionmentioning

confidence: 99%

Risk of cumulative toxicity after complete melanoma response with pembrolizumab

Hsieh¹,

Faithfull

Brown

2017

BMJ Case Reports

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Pembrolizumab is an approved first-line systemic therapy for unresectable metastatic melanoma. Despite the achievement of complete and durable responses in a small subgroup of patients, it is standard practice that pembrolizumab therapy continues beyond complete response. Nevertheless, the incidence of immune-related toxicities gradually increases with continuing pembrolizumab therapy. We report a case highlighting the occurrence of serious induced immune-related adverse events, which were attributed to pembrolizumab in a patient with metastatic melanoma who obtained a complete response (CR) after receiving pembrolizumab for a total of 6.5 months. Although mild pembrolizumab-related toxicity persists, the patient remains disease-free 5.5 months after discontinuation of pembrolizumab. Accordingly, we believe that cessation of pembrolizumab should be considered in patients who achieve a CR because of the ongoing risk of toxicity with extended pembrolizumab administration.

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Characterization of a Subtype of Autoimmune Encephalitis With Anti–Contactin-Associated Protein-like 2 Antibodies in the Cerebrospinal Fluid, Prominent Limbic Symptoms, and Seizures

Cited by 161 publications

References 19 publications

“Autoimmune Epilepsy”: Encephalitis with Autoantibodies for Epileptologists

“Autoimmune Epilepsy”: Encephalitis with Autoantibodies for Epileptologists

Autoimmune encephalitis in psychiatric institutions: current perspectives

Risk of cumulative toxicity after complete melanoma response with pembrolizumab

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