1. Acquired Neuromyotonia in Childhood: Case Report and Review
- Author
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Gabriel Gonzalez, María Eugenia Russi, Cristina Scavone, Ana Nuñez, and Graciela Barros
- Subjects
Male ,Spasm ,Pathology ,medicine.medical_specialty ,Adolescent ,Neural Conduction ,Neuromuscular Junction ,Pseudomyotonia ,Electromyography ,ELECTROMYOGRAPHIC FINDINGS ,Neuromuscular junction ,Diagnosis, Differential ,Developmental Neuroscience ,medicine ,Humans ,Motor Neuron Disease ,Muscle, Skeletal ,Autoantibodies ,Motor Neurons ,medicine.diagnostic_test ,business.industry ,Age Factors ,Muscle stiffness ,Acquired Neuromyotonia ,Carbamazepine ,Treatment Outcome ,medicine.anatomical_structure ,Neurology ,Potassium Channels, Voltage-Gated ,Peripheral nervous system ,Pediatrics, Perinatology and Child Health ,Anticonvulsants ,Isaacs Syndrome ,Neurology (clinical) ,Differential diagnosis ,business ,Neuroscience - Abstract
Recently characterized as an immune-mediated channelopaty, Isaacs' syndrome (also known as acquired neuromyotonia) was first described in 1961 in two men with persistent, generalized muscle stiffness, in addition to spontaneous, rapid discharges of motor-unit potentials on electromyography. In the peripheral nervous system, antibodies targeted to voltage-gated potassium channels induce hyperexcitability of motor axons, resulting in signs of muscle stiffness or of pseudomyotonia. A spontaneous burst of single motor-unit activity, and myokymic and neuromyotonic discharges, are the most characteristic features found in electromyography studies. This report describes Isaacs' syndrome in a child, in whom the diagnosis was made by clinical features of acquired, spontaneous muscle overactivity and typical electromyographic findings.
- Published
- 2008
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