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.
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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.
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