The clinical spectrum of Caspr2 antibody–associated disease

Sonderen, Agnes van; Ariño, Helena; Petit‐Pedrol, Mar; Leypoldt, Frank; Körtvelyessy, Péter; Wandinger, Klaus‐Peter; Lancaster, Eric; Wirtz, Paul W.; Schreurs, Marco W. J.; Smitt, Peter A.E. Sillevis; Graus, Francesc; Dalmau, Josep; Titulaer, Maarten J.

doi:10.1212/wnl.0000000000002917

Cited by 355 publications

(412 citation statements)

References 26 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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“…It is likely that the tumor-related triggering mechanisms are similar to those discussed above (figure 3). Tumors of the thymus are not involved in anti-NMDAR encephalitis but they often occur with other disorders (e.g., Caspr2, 26,27 AMPA receptor antibody-associated encephalitis 28 ). In these cases, an alteration of the immunoregulatory function of the thymus per se may facilitate the autoimmune response, as occurs in myasthenia gravis.…”

Section: Triggers Of Synaptic Autoimmunity: Tumorsmentioning

confidence: 99%

NMDA receptor encephalitis and other antibody-mediated disorders of the synapse

2016

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Investigations during the last 10 years have revealed a group of disorders mediated by antibodies against ion channels and synaptic receptors, which cause both neurologic and psychiatric symptoms. In this review, I discuss the process of discovery and immunologic triggers of these disorders, and use anti-NMDA receptor encephalitis to emphasize the importance of understanding the underlying physiopathologic mechanisms in those diseases. A better knowledge of these mechanisms reveals points of convergence with other disorders (e.g., schizophrenia), suggests treatment strategies beyond immunotherapy, and is helping us understand how memories are formed and retrieved. Neurology ® 2016;87:2471-2482 GLOSSARY BBB 5 blood-brain barrier; EphB2 5 Ephrin type B2 receptor; FLAIR 5 fluid-attenuated inversion recovery; HSE 5 herpes simplex encephalitis; IgG 5 immunoglobulin G; LEMS 5 Lambert-Eaton myasthenic syndrome; LTP 5 long-term potentiation; NMDAR 5 NMDA receptor.

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“…However, this lack of antibodies should be confirmed in further studies involving other cohorts of PN. Anti‐CASPR2 antibodies associate with neuromyotonia, limbic encephalitis, and Morvan syndrome in patients with or without thymoma 33. Our CASPR2‐positive patient presented a sensory‐motor neuropathy with demyelinating features in the EMG and no evidence of central nervous system involvement or neuromyotonia.…”

Section: Discussionmentioning

confidence: 62%

Antibodies against cell adhesion molecules and neural structures in paraneoplastic neuropathies

Siles

Martínez‐Hernández

Araque

et al. 2018

Ann Clin Transl Neurol

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ObjectiveParaneoplastic neurological syndromes (PNS) are rare neurological disorders in which ectopic expression of neural antigens by a tumor results in an autoimmune attack against the nervous system. Onconeural antibodies not only guide PNS diagnosis but may also help detecting underlying malignancies. Our project aims to uncover new potential antibodies in paraneoplastic neuropathies (PN).MethodsThirty‐four patients fulfilling diagnostic criteria of possible (n = 9; 26.5%) and definite (n = 25; 73.5%) PN without onconeural antibodies and 28 healthy controls were included in our study. Sera were tested for known antibodies against neural cell adhesion molecules and screened for novel IgG and IgM reactivities against nerve components: dorsal root ganglia (DRG) neurons, motor neurons, and Schwann cells. Patients showing autoantibodies against any of these cell types were used for immunoprecipitation (IP) studies.ResultsOverall, 9 (26.5%) patients showed significant reactivity against DRG neurons, motor neurons, or Schwann cells, whereas 5 (17.9%) healthy controls only showed moderate reactivity. Compared with control sera, serum samples from patients with paraneoplastic sensory‐motor neuropathies had a higher frequency of IgM antibodies against Schwann cells (0% vs. 40%; P = 0.0028). No novel antigens were identified from our IP experiments. Antibodies against the neural adhesion molecules CNTN1, NF155, NF140, NF186, NCAM1, L1CAM, and the CNTN1/CASPR1 complex were not detected in patients with PN. One (2.9%) patient with CIDP and thymoma had CASPR2 antibodies.InterpretationAlmost 30% of patients with PN harbor antibodies targeting neural structures, suggesting that novel neoplasm‐associated antigens remain to be discovered.

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The clinical spectrum of Caspr2 antibody–associated disease

Cited by 355 publications

References 26 publications

“Autoimmune Epilepsy”: Encephalitis with Autoantibodies for Epileptologists

“Autoimmune Epilepsy”: Encephalitis with Autoantibodies for Epileptologists

NMDA receptor encephalitis and other antibody-mediated disorders of the synapse

Antibodies against cell adhesion molecules and neural structures in paraneoplastic neuropathies

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