Transient weakness and compound muscle action potential decrement in myotonia congenita
Corresponding Author
Feza Deymeer MD, MS
Department of Neurology, Istanbul Faculty of Medicine, University of Istanbul, Çapa, 34390 Istanbul, Turkey
Department of Neurology, Istanbul Faculty of Medicine, University of Istanbul, Çapa, 34390 Istanbul, TurkeySearch for more papers by this authorSevinnç Çakirkaya MD
Department of Neurology, Istanbul Faculty of Medicine, University of Istanbul, Çapa, 34390 Istanbul, Turkey
Search for more papers by this authorPiraye Serdaroğlu MD
Department of Neurology, Istanbul Faculty of Medicine, University of Istanbul, Çapa, 34390 Istanbul, Turkey
Search for more papers by this authorLothar Schleithoff PhD
Department of Physiology, University of Ulm, 89069 Ulm, Germany
Search for more papers by this authorFrank Lehmann-Horn MD
Department of Physiology, University of Ulm, 89069 Ulm, Germany
Search for more papers by this authorReinhardt Rüdel PhD
Department of Physiology, University of Ulm, 89069 Ulm, Germany
Search for more papers by this authorCoşkun Özdemir MD
Department of Neurology, Istanbul Faculty of Medicine, University of Istanbul, Çapa, 34390 Istanbul, Turkey
Search for more papers by this authorCorresponding Author
Feza Deymeer MD, MS
Department of Neurology, Istanbul Faculty of Medicine, University of Istanbul, Çapa, 34390 Istanbul, Turkey
Department of Neurology, Istanbul Faculty of Medicine, University of Istanbul, Çapa, 34390 Istanbul, TurkeySearch for more papers by this authorSevinnç Çakirkaya MD
Department of Neurology, Istanbul Faculty of Medicine, University of Istanbul, Çapa, 34390 Istanbul, Turkey
Search for more papers by this authorPiraye Serdaroğlu MD
Department of Neurology, Istanbul Faculty of Medicine, University of Istanbul, Çapa, 34390 Istanbul, Turkey
Search for more papers by this authorLothar Schleithoff PhD
Department of Physiology, University of Ulm, 89069 Ulm, Germany
Search for more papers by this authorFrank Lehmann-Horn MD
Department of Physiology, University of Ulm, 89069 Ulm, Germany
Search for more papers by this authorReinhardt Rüdel PhD
Department of Physiology, University of Ulm, 89069 Ulm, Germany
Search for more papers by this authorCoşkun Özdemir MD
Department of Neurology, Istanbul Faculty of Medicine, University of Istanbul, Çapa, 34390 Istanbul, Turkey
Search for more papers by this authorAbstract
Twenty-five Turkish patients with recessive myotonia congenita (RMC), 16 of whom had genetic confirmation, were studied. Nineteen had transient weakness. In the upper extremities, onset age of transient weakness was usually in the early teens. All untreated RMC patients had a compound muscle action potential decrement of ⩾25%, usually above 50%, with repetitive nerve stimulation at 10/s for 5 s. Patients with other nondystrophic diseases with myotonia, except 1 patient with dominant myotonia congenita, had no transient weakness and a CMAP decrement below 25%. © 1998 John Wiley & Sons, Inc. Muscle Nerve 21:1334–1337, 1998.
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