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 journalArticle

Abstract

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.

LanguageEnglish
JournalDevelopmental Medicine and Child Neurology
Early online date6 Feb 2019
DOIs
Publication statusPublished - 6 Feb 2019

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Contactins
Isaacs Syndrome
Leucine
Glioma
Immunotherapy
Antibodies
Proteins
Peripheral Nerves
Contactin 1
Myokymia
Sodium Channel Blockers
Pediatrics
Muscle Weakness
Acute Pain
Electromyography
Blood Proteins
Lower Extremity
Therapeutics

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 https://doi.org/10.1111/dmcn.14179.  This article may be used for non-commercial purposes in accordance With Wiley Terms and Conditions for self-archiving.

Funding: Epilepsy Research UK

Cite this

Surana, Snehal ; Kumar, Ratna ; Pitt, Matthew ; Hafner, Patricia ; Mclellan, Ailsa ; Davidson, Joyce ; Prabakhar, Prab ; Vincent, Angela ; Hacohen, Yael ; Wright, Sukhvir. / Acquired neuromyotonia in children with CASPR2 and LGI1 antibodies. In: Developmental Medicine and Child Neurology. 2019.
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Surana, S, Kumar, R, Pitt, M, Hafner, P, Mclellan, A, Davidson, J, Prabakhar, P, Vincent, A, Hacohen, Y & Wright, S 2019, 'Acquired neuromyotonia in children with CASPR2 and LGI1 antibodies' Developmental Medicine and Child Neurology. https://doi.org/10.1111/dmcn.14179

Acquired neuromyotonia in children with CASPR2 and LGI1 antibodies. / Surana, Snehal; Kumar, Ratna; Pitt, Matthew; Hafner, Patricia; Mclellan, Ailsa; Davidson, Joyce; Prabakhar, Prab; Vincent, Angela; Hacohen, Yael; Wright, Sukhvir.

In: Developmental Medicine and Child Neurology, 06.02.2019.

Research output: Contribution to journalArticle

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