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Transient weakness and compound muscle action potential decrement in myotonia congenita

✍ Scribed by Feza Deymeer; Sevinnç Çakirkaya; Piraye Serdaroğlu; Lothar Schleithoff; Frank Lehmann-Horn; Reinhardt Rüdel; Coşkun Özdemir

Publisher: John Wiley and Sons
Year: 1998
Tongue: English
Weight: 94 KB
Volume: 21
Category: Article
ISSN: 0148-639X
DOI: 10.1002/(sici)1097-4598(199810)21:10<1334::aid-mus16>3.0.co;2-1

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✦ Synopsis

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 ജ25%, 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%.

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