Acquired neuromyotonia in children with CASPR2 and LGI1 antibodies

Snehal Surana, Ratna Kumar, Matthew Pitt, Patricia Hafner, Ailsa Mclellan, Joyce Davidson, Prab Prabakhar, Angela Vincent, Yael Hacohen, Sukhvir Wright

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)1344-1347
Number of pages4
JournalDevelopmental Medicine and Child Neurology
Issue number11
Early online date6 Feb 2019
Publication statusPublished - 1 Nov 2019

Bibliographical note

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


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