Transient weakness and compound muscle action potential decrement in myotonia congenita |
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Authors: | Feza Deymeer,Sevinnç Ç akirkaya,Piraye Serdaroğ lu,Lothar Schleithoff,Frank Lehmann-Horn,Reinhardt Rü del,Coş kun Ö zdemir |
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Abstract: | 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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Keywords: | myotonia congenita recessive generalized myotonia transient weakness repetitive nerve stimulation channelopathies |
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