Neuromyotonia.
Neuromyotonia is a rare condition of spontaneous and continuous muscle fibre activity of peripheral nerve origin. It represents the more severe phenotype of peripheral nerve hyperexcitability, and when acquired is often associated with antibodies to voltage-gated potassium channels. There are no specific published electromyographic or clinical diagnostic criteria for this disorder. This review highlights the classical clinical, electrophysiological and immunological features of this disorder from what is currently known in the literature to date, and also from the author's own patients' studies. Neuromyotonia is best classified as a moderately severe disorder of peripheral nerve hyperexcitability, with electromyographic features of spontaneous, continuous, irregularly occurring doublet, or multiplet single motor unit (or partial motor unit) discharges, firing at a high intraburst frequency (30-300Hz). Invariably, patients develop persistent muscle contraction, often worse following exercise. About 40% of patients with acquired neuromyotonia will have detectable voltage-gated potassium-channel antibodies. Clinical, electrophysiological and immunological measurements are important in defining the phenotype of neuromyotonia, and other, milder forms of peripheral nerve hyperexcitability.
Maddison
Paul
P
University of Oxford Department of Clinical Neurology, Radcliffe Infirmary, Oxford, OX2 6HE, United Kingdom. paul.maddison@nhs.net
D000818:Animals; D001327:Autoimmune Diseases / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D003937:Diagnosis, Differential; D004594:Electrophysiology; D006801:Humans; D020386:Isaacs Syndrome / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D018482:Muscle, Skeletal / Q000294:innervation / Q000473:pathology / Q000502:physiology