Muscle & nerve · 2018
Serial Stimulated Jitter Analysis In Juvenile Myasthenia Gravis.
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Bhatia Shivani, Quinlan Haley, McCracken Courtney, Price Eric W, Guglani Lokesh, Verma Sumit
Аннотация
INTRODUCTION: Clinical and electrophysiological studies to measures disease activity in juvenile myasthenia gravis (JMG) are limited.
METHODS: Retrospective review of the clinical profile, Myasthenia Gravis Foundation of America (MGFA) scores, serial stimulated jitter analysis (Stim-JA) of the orbicularis oculi muscle, grip strength, and spirometry of patients with JMG who were followed in a multidisciplinary clinic was performed.
RESULTS: Thirteen patients with JMG (9 females) with mean age of 13.2 ± 4.8 years and follow-up duration of 25.3 ± 8.3 months (range, 6-39) with ≥ 2 Stim-JA recordings were included. The mean jitter, mean percentage of apparent single-fiber action potentials (%ASFAP) with increased jitter, and mean %ASFAP with blocking at baseline values (77.3 ± 54.7 µs, 64.3% ± 35.8%, 39% ± 38.6%, respectively) and at follow-up (53 ± 45.4 µs, 51.2% ± 34.5%, 17% ± 29.4%, respectively) were abnormal; however, no statistically significant interval difference was noted. The electrophysiological data correlated significantly with Myasthenia Gravis Foundation of America (MGFA) class. Grip strength and spirometry did not correlate with MGFA class.
DISCUSSION: Stimulated jitter values are sensitive biomarkers in JMG. Muscle Nerve 58: 729-732, 2018.
METHODS: Retrospective review of the clinical profile, Myasthenia Gravis Foundation of America (MGFA) scores, serial stimulated jitter analysis (Stim-JA) of the orbicularis oculi muscle, grip strength, and spirometry of patients with JMG who were followed in a multidisciplinary clinic was performed.
RESULTS: Thirteen patients with JMG (9 females) with mean age of 13.2 ± 4.8 years and follow-up duration of 25.3 ± 8.3 months (range, 6-39) with ≥ 2 Stim-JA recordings were included. The mean jitter, mean percentage of apparent single-fiber action potentials (%ASFAP) with increased jitter, and mean %ASFAP with blocking at baseline values (77.3 ± 54.7 µs, 64.3% ± 35.8%, 39% ± 38.6%, respectively) and at follow-up (53 ± 45.4 µs, 51.2% ± 34.5%, 17% ± 29.4%, respectively) were abnormal; however, no statistically significant interval difference was noted. The electrophysiological data correlated significantly with Myasthenia Gravis Foundation of America (MGFA) class. Grip strength and spirometry did not correlate with MGFA class.
DISCUSSION: Stimulated jitter values are sensitive biomarkers in JMG. Muscle Nerve 58: 729-732, 2018.