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33. SFEMG in diseases
- 1. SFEMGSFEMG in diseasesin diseases MG, reinnervation, myopathyMG, reinnervation, myopathy E StålbergE Stålberg Stålberg
- 3. Single fiber action potentials inSingle fiber action potentials in Myasthenia GravisMyasthenia Gravis Stålberg Stålberg
- 4. Definition of an abnormal study • Mean MCD of 20 recordings above limits or • > 2/20 of ind pairs above “outlier” limits • Mean MCD of 20 recordings above limits or • > 2/20 of ind pairs above “outlier” limits Stålberg
- 5. Normal jitter values with voluntary activation Stålberg
- 6. Criteria of Abnormality in EDCCriteria of Abnormality in EDC 762 Patients with MG762 Patients with MG Mean JitterMean Jitter PairsPairs withwith JitterJitter
- 7. Jitter in MG (EDC muscle)Jitter in MG (EDC muscle) Stålberg
- 8. Intramuscular stimulation andIntramuscular stimulation and SFEMG recordingSFEMG recording Stålberg Stålberg
- 9. Two examples of simultaneous direct muscle fiber and axonal stimulation direct Stålberg
- 10. Effect of stim frequency in MG Stålberg
- 11. Stim-SFEMG - results inStim-SFEMG - results in MGMG Stålberg
- 12. 1 Hz 5 Hz 20 Hz10 Hz 1 Hz 5 Hz 20 Hz10 Hz 1 Hz 5 Hz 20 Hz10 Hz Abnormality in n-m transmissionAbnormality in n-m transmission Postsynaptic Presynaptic MG LEMS BTx ALSALS Organophosphate?Organophosphate? Congenital MG?Congenital MG? Arimura, K et al. 1993 jitter Pre+postsynaptic Stålberg
- 13. STIM SFEMG can be used to study • muscle membrane parameters • n-m transmission • axonal conduction • spinal reflexes • central pathways Stålberg Stålberg
- 14. Sensitivity of initial tests 550 untreated MG patients %Abnormal SFSF ANYANY SFSF EDCEDC SFSF ANYANY SFSF EDCEDCRNSRNS RNSRNSARAARA ARAARA Courtesy Sanders, unpublished Stålberg
- 15. SFEMG in MG Arm vs Face Muscles • >95% have increased jitter in either. • 70% have increased jitter in both. • 20% have increased jitter in face only. • <5% have increased jitter in arm only. • 80% have greater abnormality in face than arm. Courtesy Sanders, unpublished Stålberg
- 16. Initial jitter in arm & faceInitial jitter in arm & faceMeanMCD, µSec Courtesy Sanders, unpublished Stålberg
- 17. Sanders, Massey and Howard. Unpublished, with permission. Comparison of diagnostic tests in 550Comparison of diagnostic tests in 550 untreated Myastenia Gravis patientsuntreated Myastenia Gravis patients Ocular Generalized SFEMG EDC RNS AchR-Ab 97 99 60 89 48 76 55 80 SFEMG any muscle
- 18. EDX in MG; start with jitterEDX in MG; start with jitter analysis if availableanalysis if available Jitter analysis normal STOP Logics: we have ”never” seen normal jitter and abnormal RNS Jitter analysis abnormal RNS Why RNS in this case: to see specific patterns (LEMS, cong MGs, other disorders, to follow over time
- 19. RNS in MG if jitter analysis notRNS in MG if jitter analysis not is availableis available Why RNS: for diagnosis of MG to see specific patterns (LEMS, cong MGs, ...) to follow over time RNS in (delt), trapezius, anconeus, nasalis, clinically weak muscle 3 Hz at rest, after 10” act, and after 1´
- 21. Two types of reinnervationTwo types of reinnervation After complete denervation After partial denervation Site of lesionSite of lesion StålbergStålberg
- 22. Collateral sproutingCollateral sprouting Stålberg 2 motor units Effect of collateral sproutingStålberg
- 24. FD - 95% upper normal limitsFD - 95% upper normal limits Age Age FD FD m Ext dig comm m frontalis Stålberg
- 25. FD in patients with MGFD in patients with MG Stålberg
- 26. Concomitant block/jitterConcomitant block/jitter Stålberg, Trontelj Stålberg
- 27. FD in frontalis muscleFD in frontalis muscle after partial facial nerve lesionafter partial facial nerve lesion J. M. Massey and D. B. SandersStålberg
- 28. Stålberg
- 29. Conc EMG in reinnervationConc EMG in reinnervation StålbergStålberg
- 30. MUPs in MND, jiggleMUPs in MND, jiggle StålbergStålberg
- 31. Jiggle display in normal and in ALS Stålberg
- 32. 1 ms/div superimposed raster filled Jiggle in reinnervation Stålberg
- 33. Raster and filled to show the Jiggle Stålberg
- 34. The jiggle Expressed as Mean of consecutive amplitude differences (CAD) or Mean of consecutive correlation coefficients (CCC) Stålberg
- 36. Stålberg FD in myopathy and in ALSFD in myopathy and in ALS Stålberg
- 37. AA BB CC DD EE focal lesion splitting neurogenic lesion local hyperexitability ephaptic transmission THE DYSTROPHIC MOTOR UNITTHE DYSTROPHIC MOTOR UNIT degeneration (satellite cell) Stålberg
- 38. FD Jitter Myopathy Inactive reinnervation Ongoing reinnervation Myasthenia Gravis FD and jitter in different disordersFD and jitter in different disorders Stålberg Stålberg
- 39. SFEMG INDICATIONS •Neuromuscular transmission in diseasesNeuromuscular transmission in diseases •Experimental studies of n-m transmissionExperimental studies of n-m transmission •Spatial organisation of MUs in diseasesSpatial organisation of MUs in diseases •Firing patternFiring pattern •Spike triggeringSpike triggering •Propagation velocityPropagation velocity StålbergStålberg
Editor's Notes
- Textual summary of percent positive dx tests in ocularand generalized MG.
- Analysis of initial SFEMG studies performed in EDC (532 pts) and Frontalis (339 pts) in acquired MG.