Premium
Acquired neuromyotonia in children with CASPR 2 and LGI 1 antibodies
Author(s) -
Surana Snehal,
Kumar Ratna,
Pitt Matthew,
Hafner Patricia,
Mclellan Ailsa,
Davidson Joyce,
Prabakhar Prab,
Vincent Angela,
Hacohen Yael,
Wright Sukhvir
Publication year - 2019
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/dmcn.14179
Subject(s) - neuromyotonia , medicine , immunotherapy , myokymia , weakness , sodium channel blocker , antibody , electromyography , immunology , sodium channel , cancer , surgery , physical medicine and rehabilitation , sodium , chemistry , organic chemistry
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 ( LGI 1) and contactin‐associated protein‐like 2 ( CASPR 2) have been reported. We describe three paediatric patients with acquired neuromyotonia and CASPR 2 and LGI 1 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.