Clinical manifestations of patients with CASPR2 antibodies

Jun-Sang Sunwoo; Soon-Tae Lee; Jung-Ick Byun; Jangsup Moon; Jung-Won Shin; Da-Eun Jeong; Gun-Hee Lee; Seong Ho Jeong; Yong-Won Shin; Keun-Hwa Jung; Doo Young Lee; Daejong Jeon; Ki-Young Jung; Manho Kim; Sang Kun Lee; Kon Chu

doi:10.1016/j.jneuroim.2015.03.005

Clinical manifestations of patients with CASPR2 antibodies

J Neuroimmunol. 2015 Apr 15:281:17-22. doi: 10.1016/j.jneuroim.2015.03.005. Epub 2015 Mar 6.

Authors

Affiliations

¹ Department of Neurology, Laboratory for Neurotherapeutics, Biomedical Research Institute, Seoul National University Hospital, Korea Autoimmune Synaptic and Paraneoplastic Encephalitis Registry (KASPER), Seoul, South Korea.
² Department of Neurology, CHA University, CHA Bundang Medical Center, Seongnam, South Korea.
³ Department of Neurology, Chung-Ang University College of Medicine, Seoul, South Korea.
⁴ Department of Neurology, School of Medicine, Kyungpook National University, Daegu, South Korea.
⁵ Department of Neurology, Yonsei University College of Medicine, Seoul, South Korea.
⁶ Department of Neurology, Laboratory for Neurotherapeutics, Biomedical Research Institute, Seoul National University Hospital, Korea Autoimmune Synaptic and Paraneoplastic Encephalitis Registry (KASPER), Seoul, South Korea; Protein Metabolism Medical Research Center, College of Medicine, Seoul National University, Seoul, South Korea.
⁷ Department of Neurology, Laboratory for Neurotherapeutics, Biomedical Research Institute, Seoul National University Hospital, Korea Autoimmune Synaptic and Paraneoplastic Encephalitis Registry (KASPER), Seoul, South Korea. Electronic address: stemcell.snu@gmail.com.

PMID: 25867463
DOI: 10.1016/j.jneuroim.2015.03.005

Abstract

Contactin-associated protein-like 2 (CASPR2) is one of the target antigens of voltage-gated potassium channels (VGKC) complex antibodies. There has been relatively little information in the literature regarding CASPR2 autoimmunity, especially in Asian population. We investigated the presence of CASPR2 antibodies in patients with presumed autoimmune neurological disorders and described the clinical features, laboratory findings, and responses to immunotherapy. Five patients were identified to be positive for CASPR2 antibodies. The results obtained here suggested that CASPR2 antibodies might be the possible cause of epilepsy even in the absence of typical features of limbic encephalitis and that immunotherapy could provide a favorable outcome.

Keywords: CASPR2; Epilepsy; Immunotherapy; Neuromyotonia.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adult
Aged
Autoantibodies / blood*
Autoimmune Diseases of the Nervous System / blood*
Autoimmune Diseases of the Nervous System / diagnosis
Autoimmune Diseases of the Nervous System / immunology
Autoimmunity / physiology*
Child
Epilepsy / blood*
Epilepsy / diagnosis
Epilepsy / immunology
Female
Follow-Up Studies
HEK293 Cells
Humans
Male
Membrane Proteins / blood*
Middle Aged
Nerve Tissue Proteins / blood*

Substances

Autoantibodies
CNTNAP2 protein, human
Membrane Proteins
Nerve Tissue Proteins