𝔖 Bobbio Scriptorium
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Myasthenic symptoms in patients with mitochondrial myopathies

✍ Scribed by Nadine Le Forestier; Pr. Romain K. Gherardi; Christian Meyrignac; Djillali Annane; Cécile Marsac; Francoise Gray; Philippe Gajdos

Publisher
John Wiley and Sons
Year
1995
Tongue
English
Weight
243 KB
Volume
18
Category
Article
ISSN
0148-639X

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✦ Synopsis

Mitochondria1 myopathies (MM) include chronic progressive external ophthalmoplegia (CPEO), Kearns-Sayre syndrome (KSS), or various encephalomyopathies.' Ptosis or weakness increasing with muscle activity suggesting myasthenia gravis (MG) have been occasionally described in patients with MM.2,4,72103' Successful anticholinesterase therapy was reported once,' decrement of the com- pound muscle action potential (CMAP) on repetitive stimulation was detected in several patients with MM,' and neuromuscular transmission defect was further suggested by single-fiber electromyography studies.*z5

We describe 3 patients with CPEO or KSS who were first thought to have MG because of ptosis, fatiguable weakness, positive neostigmine test, and electrophysiological evidence for mild to marked neuromuscular transmission block.

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