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Acquired neuromyotonia in children with CASPR2 and LGI1 antibodies

Surana, S; Kumar, R; Pitt, M; Hafner, P; Mclellan, A; Davidson, J; Prabakhar, P; ... Wright, S; + view all (2019) Acquired neuromyotonia in children with CASPR2 and LGI1 antibodies. Developmental Medicine & Child Neurology , 61 (1) pp. 1344-1347. 10.1111/dmcn.14179. Green open access

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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.

Type: Article
Title: Acquired neuromyotonia in children with CASPR2 and LGI1 antibodies
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1111/dmcn.14179
Publisher version: https://doi.org/10.1111/dmcn.14179
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Neuroinflammation
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Neurosciences Dept
URI: https://discovery.ucl.ac.uk/id/eprint/10082646
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