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Abstract Details

Stimulated Jitter Analysis for the Evaluation of Neuromuscular Junction Disorders in Children
Neuromuscular and Clinical Neurophysiology (EMG)
P7 - Poster Session 7 (8:00 AM-9:00 AM)
10-004

To evaluate Stimulated jitter analysis (Stim-JA) among children with neuromuscular junction (NMJ) disorders.

Stim-JA is a rapid neurophysiological test to evaluate NMJ transmission. It involves stimulation of the distal branch of the facial nerve with a monopolar needle electrode. Time variability of muscle fiber action potentials (jitter) is recorded from orbicularis oculi muscle using a concentric needle electrode. Stim-JA is a preferred test for children, who often have difficulty making controlled activations. However, there is a paucity of literature detailing the mean jitter and % abnormal apparent single fiber action potentials (ASFAPs) between normal and abnormal Stim-JA studies.

Retrospective analysis of 227 studies performed in the pediatric electrodiagnostic laboratory at Children’s Healthcare of Atlanta from 2014 to 2021. Mean age of the participants was 8.6 ± 5.9 years (133 girls and 94 boys). Studies having two or more ASFAPs with jitter values 34 μs and/or any blocking were categorized as abnormal. Serum anti-AchR receptor and Myasthenia Gravis Foundation of America (MGFA) score were recorded.

Thirty percent studies (n=68) with mean jitter values of 13.2 ± 4.8 μs (range 1.29-21.8 μs) were normal. Seventy percent studies (n=159) with mean jitter values of 33.5 ± 17.1 μs (range 8.33-95.6 μs) were abnormal. Difference in mean jitter was statistically significant (p<0.001). Percent abnormal ASFAP’s was significantly different between normal (2.2%, range 0-10%) and abnormal (55%, range 9-100%) studies (p<0.001). Eleven (16%) normal studies showed seropositivity while 96 (60%) of abnormal studies showed seropositivity. MGFA severity scores differed significantly between normal (mean 0.5 ± 0.8, range 0-5) and abnormal (mean 1.5 ± 0.9, range 0-5) studies (p<0.001).

We conclude that percentage abnormal ASFAPs and presence of blocking are the best indicators of an abnormal Stim-JA study. Stim-JA sensitivity 0.62 and specificity of 0.84. Study limitation is a single-center experience.
Authors/Disclosures
Vishva Natarajan (Georgia Institute of Technology)
PRESENTER
Mr. Natarajan has received personal compensation for serving as an employee of Pfizer. Mr. Natarajan has stock in Apple. Mr. Natarajan has stock in Tesla. Mr. Natarajan has stock in Microsoft. Mr. Natarajan has stock in Mind Medicine. Mr. Natarajan has received research support from Georgia Institute of Technology.
Sumit Verma, MD (Emory Children's Center) Dr. Verma has nothing to disclose.