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Guillain–Barré-like syndrome: an uncommon feature of CASPR2 and LGI1 autoimmunity

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Abstract

Contactin-associated protein-like 2 (CASPR2) and leucine-rich glioma-inactivated 1 (LGI1) are essential components of the voltage-gated Kv1 potassium channel complex and are extensively expressed in both central and peripheral nervous system. Autoimmune CASPR2 and LGI1 disorders commonly present with Morvan syndrome (Mos) and/or limbic encephalitis, but whether Guillain–Barré syndrome (GBS) is a specific clinical phenotype is unknown. Here, we first reported an adult patient with dual CASPR2 and LGI1 antibodies in both serum and cerebrospinal fluid, who initially presented with a GBS-like syndrome and developed a typical MoS and respiratory paralysis, with a rapid resolution of his neurological symptoms and disappearance of autoantibodies after treatment with plasma exchange. Additionally, we also provided an overview of the previously reported GBS cases associated with CASPR2 or LGI1 antibodies. These cases expand the phenotypic spectrum of CASPR2 and LGI1 autoimmune syndromes, implying that these two antigens, especially CASPR2, are likely to participate in the etiology of GBS as a potential new target antigen, which deserves further exploration.

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Data availability

The data sets used and/or analyzed during the current study are available from the corresponding author on reasonable request.

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Authors and Affiliations

  1. Department of Neurology, Shengjing Hospital of China Medical University, 39 Hua Xiang Road, Shenyang, 110022, Liaoning, People’s Republic of China

    Xiaoping Tan, Yang Liu, Xiaoli Wu & Yang Guo

Authors
  1. Xiaoping Tan
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  2. Yang Liu
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  3. Xiaoli Wu
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  4. Yang Guo
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Correspondence to Yang Guo.

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The authors declare that they have no conflict of interest.

Ethical standards

This study was approved by the local Institutional Review Board and performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

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Informed consent was obtained from the patient.

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Tan, X., Liu, Y., Wu, X. et al. Guillain–Barré-like syndrome: an uncommon feature of CASPR2 and LGI1 autoimmunity. J Neurol 269, 5893–5900 (2022). https://doi.org/10.1007/s00415-022-11248-y

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  • DOI: https://doi.org/10.1007/s00415-022-11248-y

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