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NEUROLOGY 1982;32:827
© 1982 American Academy of Neurology

Electromyographic distinction between paramyotonia congenita and myotonia congenita

Effect of cold

Viggo Kamp Nielsen, Mogens Laue Friis and Torsten Johnsen

Departments of Clinical Neurophysiology (Dr. Nielsen) and Otolaryngology (Dr. Johnsenl, Glostrup Hospital, University of Copenhagen, and the Department of Neurology (Dr. Friis), State University Hospital (Rigshospitalet), Copenhagen and Frederiksborg Amts Centralsygehus, Hillerød, Denmark.

We studied three patients with paramyotonia congenita and three with myotonia congenita. At room temperature, myotonic bursts in the EMG were similar in the two disorders. After repetitive maximal contractions, patients with paramyotonia showed decreasing recruitment pattern and increasing after-activity, while myotonia patients showed unchanged recruitment pattern, and after-activity tended to decrease, but these differences were not consistent. However, immersion of the hand in ice water for 10 minutes caused unambiguous differences: In paramyotonia, the myotonic discharges disappeared and the muscles went into stiff, electrically silent, contracture; in myotonia patients, the myotonic activity increased and muscle contraction patterns remained normal.

Address correspondence and reprint requests to Dr. Nielsen, Department of Neurology, University of Pittsburgh, 322 Scaife Hall, Pittsburgh, PA 15261.

The study was financially supported by "Grosserer A.V. Lykfeldt og hustrus legal."

Accepted for publication December 22, 1981.






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