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Letters to the editor

✍ Scribed by Erich W. Streib; Russell J. M. Lane; Douglass M. Turnbull; Peter Hudgson; Professor Sir John Walton; Roger A. Brumback; Jeffery W. Gerst; John Z. Heckmatt; Victor Dubowitz; Ana Lia Taratuto; Alberto Dubrovsky

Publisher: John Wiley and Sons
Year: 1984
Tongue: English
Weight: 460 KB
Volume: 7
Category: Article
ISSN: 0148-639X
DOI: 10.1002/mus.880070709

No coin nor oath required. For personal study only.

✦ Synopsis

Subramony et a1.6 used nerve conduction studies to distinguish between paramyotonia congenita (PC) and myotonia congenita (MyC). This is in agreement with our I would like to comment on several crucial points when using this technique.

Severe transient paresis with a marked decrease of the compound muscle action potential (CMAP) may be striking in autosomal recessive MyC (Fig. l), but recovery of the CMAP always occurs within seconds to minutes depending on the severity of ~e a k n e s s . ~

The fundamental and specific difference in PC compared with the other myotonic syndromes is the marked delay of CMAP-recovery, which may take hours (Fig. 2).4,5

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