From VGKC to LGI1 and Caspr2 encephalitis: The evolution of a disease entity over time
| Autor: | Marco W.J. Schreurs, Maarten J. Titulaer, A. van Sonderen, P. A. E. Sillevis Smitt, Paul W. Wirtz |
|---|---|
| Přispěvatelé: | Neurology, Immunology |
| Rok vydání: | 2016 |
| Předmět: |
0301 basic medicine
medicine.medical_treatment Immunology Central nervous system Nerve Tissue Proteins Disease 03 medical and health sciences 0302 clinical medicine Limbic Encephalitis medicine Animals Humans Immunology and Allergy Clinical significance Caspr2 Antibody Autoantibodies biology business.industry Limbic encephalitis Intracellular Signaling Peptides and Proteins VGKC Membrane Proteins Proteins Immunotherapy medicine.disease 030104 developmental biology medicine.anatomical_structure Potassium Channels Voltage-Gated biology.protein Encephalitis LGI1 Hyponatremia business 030217 neurology & neurosurgery |
| Zdroj: | Autoimmunity Reviews, 15(10), 970-974. Elsevier |
| ISSN: | 1568-9972 |
| DOI: | 10.1016/j.autrev.2016.07.018 |
| Popis: | A wide variety of clinical syndromes has been associated with antibodies to voltage-gated potassium channels (VGKCs). Six years ago, it was discovered that patients do not truly have antibodies to potassium channels, but to associated proteins. This enabled the distinction of three VGKC-positive subgroups: anti-LGI1 patients, anti-Caspr2 patients and VGKC-positive patients lacking both antibodies. Patients with LGI1-antibodies have a limbic encephalitis, often with hyponatremia, and about half of the patients have typical faciobrachial dystonic seizures. Caspr2-antibodies cause a more variable syndrome of peripheral or central nervous system symptoms, almost exclusively affecting older males. Immunotherapy seems to be beneficial in patients with antibodies to LGI1 or Caspr2, stressing the need for early diagnosis. Half of the VGKC-positive patients lack antibodies to both LGI1 and Caspr2. This is a heterogeneous group of patients with a wide variety of clinical syndromes, raising the question whether VGKC-positivity is truly a marker of disease in these patients. Data regarding this issue are limited, but a recent study did not show any clinical relevance of VGKC-positivity in the absence of antibodies to LGI1 and Caspr2. The three VGKC-positive subgroups are essentially different, therefore, the lumping term 'VGKC-complex antibodies' should be abolished. (C) 2016 The Authors. Published by Elsevier B.V. |
| Databáze: | OpenAIRE |
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