Clinical electrophysiological characterization of the acquired neuromyotonia phenotype of autoimmune peripheral nerve hyperexcitability.
Acquired autoimmune neuromyotonia is regarded as part of the spectrum of peripheral nerve hyperexcitability disorders. We aimed to use clinical neurophysiological measurements to study the extent, distribution, and characteristics of spontaneous motor unit potentials in 11 patients with acquired neuromyotonia. Investigations revealed that most spontaneous discharges recorded were motor unit, or partial motor unit potentials of normal size. Bursts of motor unit potentials arose more commonly from distal portions of the peripheral nerve and had abnormal absolute and relative refractory periods. Spontaneous discharges in some patients occurred in semirhythmic bursts in certain muscles. No patient had neurophysiological abnormalities detectable in first-order neurons of the central nervous system when using transcranial magnetic stimulation to estimate the threshold for corticomotor excitation and determine central motor conduction time. Only patients with coexistent myasthenia gravis had neurophysiologically detectable defects in neuromuscular transmission. The pathogenic region of abnormality in peripheral nerve hyperexcitability disorders therefore seems to lie within the terminal branches of peripheral motor nerves.
University Department of Clinical Neurology, Radcliffe Infirmary, Oxford, UK. paul.maddison@nhs.net
Paul
P
Maddison
Kerry R
KR
Mills
John
J
Newsom-Davis
D000200:Action Potentials; D000328:Adult; D000368:Aged; D020274:Autoimmune Diseases of the Nervous System / Q000503:physiopathology*; D004569:Electroencephalography; D004576:Electromyography; D005260:Female; D006801:Humans; D020386:Isaacs Syndrome / Q000276:immunology* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology; D009431:Neural Conduction; D009469:Neuromuscular Junction / Q000503:physiopathology; D009475:Neurons, Afferent / Q000502:physiology; D010641:Phenotype