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Oral Presentations: Joint Seminar with SFEMG/QEMG Group: Single ber EMG and quantitative EMG JS1-3 Single ber EMG in diagnosis of myasthenia gravis L. Cui1 1 Peking Union Medical College Hospital, Beijing, China Single ber electromyography (SFEMG) is a selective recording technique in which a needle electrode with a small recording surface in the side is used to identify action potentials from individual muscle bers. The SFEMG parameters of greatest value are jitter and ber density (FD). Jitter objectively reects the transmission function of individual neuromuscular junctions and helps to diagnose neuromuscular junction diseases. Objective: To review SFEMG clinical uses and evaluate the sensitivity of SFEMG, repetitive nerve stimulation RNS in diagnosis of myasthenia gravis. Methods: One hundred and seven patients with OMG and 80 healthy controls were recruited. SFEMG was performed in extensor digitorum communis (EDC) and frontailis. Result: In healthy controls, the mean jitter was 27.68.2 ms, the percentage of jitter >55 ms was 0, the percentage of block was 0 in EDC. In patients with OMG, The mean jitter was 41.713.5 ms in EDC and 65.427.6 ms in Frontalis (P < 0.01), the percentage of jitter >55 ms was 17.9%15.1% in EDC and 34.5% 27.8% in frontalis (P < 0.01). The percentage of blocking was 5.5%4.6% in EDC and 29.9%21.3% in frontalis (P < 0.01). 63.1% patients with OMG showed abnormal SFEMG in EDC and 85.2% in frontalis (P < 0.01). 29.8% patents with OMG showed abnormal RNS which is signicantly lower than SFEMG. Increased mean jitter and the percentage of blocking was negatively correlated with maximum decrement of RNS (r = 0.579, P < 0.01). Mean jitter, the percentage of jitter >55 ms, the percentage of block were signicantly higher in patients with abnormal RNS than those with normal RNS. 102 patients were followed up 5 years. The results of SEMG is not correlated to the Osserman Classicaltion. Conclusion: SFEMG is more sensitive than RNS and AchRAb in diagnosis of OMG, especially when recorded in frontalis. RNS, AchRAb and SFEMG are complementary for the diagnosis of OMG and is not the marker of prognosis. JS1-4 Single ber EMG in MuSK antibody positive MG
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Joint Seminar with SFEMG/QEMG Group: Single ber EMG and quantitative EMG
Part I JS1-1 Jitter with concentric electrode: pitfalls and results
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E.V. Stalberg1 Dept Clinical Neurophysiology, Uppsala University Hospital, Sweden

Due to discouraged use of re-usable material in medicine, alternatives to SFEMG electrodes have been explored for jitter measurements. Small facial concentric needle electrodes (CNE) and proper signal ltering may give relatively selective recordings. High pass lter of 500 to 2000 Hz have been tried in different laboratories. We have found that 1000 Hz gives an optimal balance between effective removing slow wave components and still allowing for visual inspection of irregularities in the signals, indicating summation. The denition of acceptable signals is more difcult for CN than for SFEMG recordings. The spike should have a sharp rising phase, without notches or shoulders and be constant on consecutive discharges, best seen in superimpositions of traces. The time measurements can be made either between two points dened by a voltage level or between peaks of the signals. The latter is better since it is not much inuenced by the riding of signals on each other, and since it can simultaneously record all spikes in a multispike recording, without reanalysis.CN recordings have also been used with electrical activation in healthy controls and in children. This is more demanding than with voluntary activation for technical reasons, with more risk of summation. Reference values for voluntary and stimulated jitter have been collected for healthy controls in a few laboratories. The CN jitter with voluntary activation is 3 5 msec less than with SFEMG electrodes. With electrical stimulation the values are more similar for the two methods. In yet small material of comparison the methods in pathology (myasthenia), the diagnostic sensitivity is similar. Caution should be observed to declare a CNE study abnormal or normal in situations of borderline jitter values. More reference values must be collected from multicenter studies, after agreement on lter settings, signal denitions and analysis methods. JS1-2 Jitter analysis in children M.C. Pitt1 1 Department of Clinical neurophysiology, Great Ormond Street Hospital for Chidren NHS Trust, London, UK There is a need for a neurophysiological screening test for neuromuscular transmission disorders (NTD) in children. The test should not need special preparation such as general anaesthesia or sedation. With this children who may have myasthenic conditions which are rare, potentially fatal, in some cases, but treatable can be identied and further investigation more effectively targeted. Repetitive nerve stimulation (RNS) and single bre electromyography (SFEMG) from experience in adults with autoimmune myasthenia gravis (AIMG) offer the most potential. RNS is painful and is not positive unless block of NMT is present and is unsuitable as a screening test. Volitional SFEMG is not possible under the age of 8 years. Stimulation SFEMG of orbicularis oculi was chosen. SF needles are not used for a variety of reasons. Concentric needle electrodes (CNE), 30 gauge, offer an alternative with comparable results. The exercise now more accurately described as Jitter Analysis with Concentric needle Electrodes (JACE). Over 97% are accomplished successfully with local anaesthesia only. The selection of action potentials suitable for calculation of jitter presents real difculty once abnormality is present because of superimposition and phase cancellation. Other means of recognizing abnormal jitter are proposed. Meticulous attention to technique and in particular the threshold for stimulation is crucial to the effectiveness of the test. With experience in over 470 cases ranging from neonates to 18 years of age, sensitivity and negative predictive value for the diagnosis of myasthenic are both around 95%. Specicity is more modest nearer 50% reecting the fact that other conditions are associated with NTD including some myopathies and bulbar palsy. It is now used freely by clinicians and an exponential increase in usage has taken place over the last three years changing from around 15 to 120 per annum. Unusual and unexpected presentations of myasthenia are being discovered.

D.B. Sanders1 Duke University School of Medicine, Durham, NC, USA

Antibodies to muscle specic receptor tyrosine kinase (MuSK) are found in 40% to 50% of patients with generalized myasthenia gravis who lack antibodies to the acetylcholine receptor (AChRab). Weakness and electrodiagnostic abnormalities in MuSK antibody positive MG (MMG) may be distributed in a pattern different from non-MuSK MG, which can make it difcult to suspect or conrm the diagnosis, clinically or electrodiagnostically. Repetitive nerve stimulation (RNS) and jitter studies are usually more abnormal in the face than the limbs, as in non-MuSK MG, but the overall diagnostic sensitivity of RNS is less than in non-MuSK MG. SFEMG is the most sensitive electrodiagnostic procedure in MMG if muscles most likely to be involved are examined. For example, in MMG patients with predominantly facial and pharyngeal muscle weakness, jitter may be normal in limb muscles but markedly abnormal in facial muscles. Overall, we have found increased jitter in at least one muscle in all of our MMG patients, but in the extensor digitorum in only 50%, compared to 80% of AChRab-negative and 91% of AChRab-positive patients. In MMG patients with weakness predominantly in proximal and axial muscles, jitter may be normal in limb and facial muscles, but markedly abnormal in a weak neck or shoulder muscle. Thus, the physiologic abnormality in MMG is not as diffusely distributed as in non-MuSK MG, and it is important to recognize that electrodiagnostic abnormalities may not be found in the muscles that are usually examined for MG. The potentially more limited distribution of physiologic abnormalities should also be considered when performing microphysiologic studies in MMG: abnormalities might not be seen in the muscles in which these studies are usually performed in non-MuSK MG.