Acquired neuromyotonia in children with CASPR2 and LGI1 antibodies.

Journal: Developmental Medicine And Child Neurology
Treatment Used: Sodium channel blockers
Number of Patients: 3
MediFind Summary

Summary: The case discusses three children with acquired neuromyotonia.

Conclusion: Sodium channel blockers may be used to treat acquired neuromyotonia in children.


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.

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

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