O2-018 Effect of compound muscle action potential after peripheral nerve stimulation normalization on anesthetic fade of intraoperative transcranial motor-evoked otential

2020 ◽  
Vol 131 (10) ◽  
pp. e261-e262
Author(s):  
Satoshi Tanaka ◽  
Tomoko Watanabe ◽  
Jiro Akimoto ◽  
Junko Takanashi ◽  
Hidehiro Oka
2004 ◽  
Vol 19 (3) ◽  
pp. 175-182 ◽  
Author(s):  
Christina A. Gurnett ◽  
Judy A. Bodnar ◽  
Jeffrey Neil ◽  
Anne M. Connolly

We report 10 children with congenital myasthenic syndromes diagnosed by clinical features, electrodiagnostic studies, and response to acetylcholinesterase inhibitors. Age at diagnosis (mean = 4.4 years; range 0.2–10 years) correlated with age fatigue was recognized. Symptoms at presentation included mild gross motor development delay (7/10), speech articulation difficulty (5/10), and respiratory and feeding difficulties resulting in poor growth in 7 of 10 children. None of the five children with possible presynaptic abnormalities had decremental compound muscle action potential responses to 2 Hz repetitive nerve stimulation. Instead, electrodiagnostic studies showed a more than 100% increment of compound muscle action potential amplitude during 50 Hz repetitive nerve stimulation in two children and sustained compound muscle action potential decrement to 2 Hz repetitive nerve stimulation after depletion (10 Hz stimulation for 10 min) in four children. Muscle biopsies ( n = 7) showed mild to severe variation in fiber size. Our experience suggests that many children with congenital myasthenic syndromes might be undiagnosed because of atypical presentation and because additional electrophysiologic studies are required.


Gerontology ◽  
1999 ◽  
Vol 45 (3) ◽  
pp. 168-173 ◽  
Author(s):  
Katsumi Kurokawa ◽  
Yasuyo Mimori ◽  
Eiji Tanaka ◽  
Tatsuo Kohriyama ◽  
Shigenobu Nakamura

2004 ◽  
Vol 19 (3) ◽  
pp. 175-182
Author(s):  
Christina A. Gurnett ◽  
Judy A. Bodnar ◽  
Jeffrey Neil ◽  
Anne M. Connolly

We report 10 children with congenital myasthenic syndromes diagnosed by clinical features, electrodiagnostic studies, and response to acetylcholinesterase inhibitors. Age at diagnosis (mean = 4.4 years; range 0.2–10 years) correlated with age fatigue was recognized. Symptoms at presentation included mild gross motor development delay (7/10), speech articulation difficulty (5/10), and respiratory and feeding difficulties resulting in poor growth in 7 of 10 children. None of the five children with possible presynaptic abnormalities had decremental compound muscle action potential responses to 2 Hz repetitive nerve stimulation. Instead, electrodiagnostic studies showed a more than 100% increment of compound muscle action potential amplitude during 50 Hz repetitive nerve stimulation in two children and sustained compound muscle action potential decrement to 2 Hz repetitive nerve stimulation after depletion (10 Hz stimulation for 10 min) in four children. Muscle biopsies ( n = 7) showed mild to severe variation in fiber size. Our experience suggests that many children with congenital myasthenic syndromes might be undiagnosed because of atypical presentation and because additional electrophysiologic studies are required.


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