Acquired neuromyotonia in children with CASPR2 and LGI1 antibodies


Acquired neuromyotonia is a form of peripheral nerve hyperexcitability. In adults, pathogenic antibodies that target the extracellular domains of leucine-rich glioma-inactivated protein 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) have been reported. We describe three paediatric patients with acquired neuromyotonia and CASPR2 and LGI1 serum antibodies. They all presented with acute-onset myokymia and pain in the lower limbs; one patient also had muscle weakness. Electromyography was suggestive of peripheral nerve hyperexcitability. Two patients improved without immunotherapy; one treated patient remained immunotherapy-dependent. Although not fatal, acquired paediatric neuromyotonia can be disabling. It is amenable to symptomatic treatment or may undergo spontaneous recovery. More severe cases may require rational immunotherapy. What this paper adds: The symptoms of neuromyotonia may resolve spontaneously or may require sodium channel blockers. Patients with debilitating symptoms who are refractory to symptomatic therapy may require immunotherapy.

Publication DOI:
Divisions: College of Health & Life Sciences
Additional Information: This is the peer reviewed version of the following article: Surana, S. , Kumar, R. , Pitt, M. , Hafner, P. , Mclellan, A. , Davidson, J. , Prabakhar, P. , Vincent, A. , Hacohen, Y. and Wright, S. (2019), Acquired neuromyotonia in children with CASPR2 and LGI1 antibodies. Dev Med Child Neurol., which has been published in final form at  This article may be used for non-commercial purposes in accordance With Wiley Terms and Conditions for self-archiving. Funding: Epilepsy Research UK
Uncontrolled Keywords: Pediatrics, Perinatology, and Child Health,Developmental Neuroscience,Clinical Neurology
Publication ISSN: 1469-8749
Last Modified: 03 Jun 2024 07:37
Date Deposited: 30 Jan 2019 15:50
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Related URLs: https://onlinel ... 1111/dmcn.14179 (Publisher URL)
http://www.scop ... tnerID=8YFLogxK (Scopus URL)
PURE Output Type: Article
Published Date: 2019-11-01
Published Online Date: 2019-02-06
Accepted Date: 2018-12-22
Authors: Surana, Snehal
Kumar, Ratna
Pitt, Matthew
Hafner, Patricia
Mclellan, Ailsa
Davidson, Joyce
Prabakhar, Prab
Vincent, Angela
Hacohen, Yael
Wright, Sukhvir (ORCID Profile 0000-0002-5464-3779)



Version: Accepted Version

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