1. Electrodiagnostic Testing
MAJ Min Ho Chang MD

2. Outline What is EDX? Anatomy & Physiology Nerve Injury
Nerve Conduction Studies/Needle Exam
Clinical Utility

3. EMG and Nerve Conduction Studies
An extension of the Physical Examination
Assess physiology of nerve and muscle
Quantitates nerve and/or muscle injury
Real time data
Provides Useful Data Regarding Nerve Injury
Diagnosis - Duration
Prognosis - Site
Treatment - Type
Further Testing - Severity
Terminology, EMG by itself does not include NCS

4. Anatomy & Physiology Motor Unit Anterior Horn Cell Axon
Terminal Branches
Neuromuscular Junction
Muscle Fibers

5. Epineurium Perineurium Endoneurium Myelin Axons
A nerve  is usually made up from a variety of fascicles . Each fascicle is encased by perineurium.  Inside the fascicle are a group of axons bathed in endoneurial fluid. The Endoneurium lines each individual axon and its myelin sheath Each axon has an insulating lining of myelin – a fatty material inside the Schwann cells. Between the fascicles is a fatty material called the interfascicular  epineurium.  The nerve is then wrapped in the main epineurium.  Even if the sensitive living axons are damaged, the conduit made up of epineurium and perineurium will often survive and provide a pathway for regrowing nerves.

6. Even few damages along nodes of ranvier can cause conduction block, saltatory conduction

7. Action Potential
What makes it excitable is the possibility of depolarization or reversal of this potential. This occurs as the result of gated channels. Gated channels have gates which can open or close to allow particular ions to pass through the channel. They respond to a stimulus such as a voltage change, chemical,  mechanical, or other stimulus which causes them to open. On the cell membrane of a skeletal muscle cell ACh causes the gates of Na+ channels to open, allowing Na+ ions to enter the cell. Because it opposes the existing polarization of ions by letting positive ions into the cell, we say it depolarizes the membrane.  Many such channels opening at once can produce a significant membrane depolarization. Membrane depolarization in turn causes opening of voltage-gated channels (Slide 9) which are sensitive to reduced membrane potential. When a certain amount of depolarization occurs, called the threshold, it results in all of the voltage-gated channels opening and produces an action potential. CFL: Critical firing level

8. Nerve Fiber Classificaton
Fiber Type
Function
Diameter
Conduction Velocity Aα
Proprioception, somatomotor, touch
10-20
50-120 Aβ
Touch, pressure, extrafusal
4-12
25-70 Aγ
Motor to muscle spindle
2-8
10-50 Aδ
Pain (esp cold)
1-5
3-30 B
Preganglionic autonomic
1-3
3-15 C
Pain/temp/postganglionic autonomic/mechanoreceptor
<1
<2

9. NMJ Anatomy
Synaptic cleft
Acetylcholine
Motor end plate

10. Electrochemical Conduction
Acetylcholine
In presynaptic vesicles (Quanta=10000)
Exocytosis via Ca++ mediated pathway

11. Muscle Fibers
Each skeletal muscle fiber has many bundles of myofilaments. Each bundle is called a myofibril. The myofilaments of a myofibril are arranged in a regular fashion so that their ends are all lined up. This is what gives the muscle its striated appearance. The contractile units of the cells are called sarcomeres. The sarcoplasmic reticulum is a specialized endoplasmic reticulum that stores calcium ions needed for muscle contraction

12. MUSCLE CONTRACTION Troponin-Tropomyosin complex Calcium Binding
Excitation-Contraction Coupling
At rest actin and myosin are prevented from contacting each other by two other proteins: tropomyosin and the Ca++ binding protein troponin. Upon stimulation, Ca++ is released from internal stores and binds to troponin which induces a conformational change of tropomyosin and allows actin-myosin interaction.
The myosin head can bind hydrolise ATP (loss of one phosphate)to ADP. This gives energy to the myosin head to bind to actin and to bend pushing the acting filaments along. ADP is then resubstituted with ATP and actin and myosin come apart. Hydrolisis of ATP will start another binding and sliding cycle.pushing the actin filament along and resulting in contraction of the cell
The transverse tubular systems arise as invaginations of the plasmalemma and ramify as an intricate network within the sarcomere. They are conduits along which the action potential is transmitted to the depths of the sarcomere. The sarcoplasmic reticulum surrounds the myofibrils and ends as terminal cisterns which contain calcium. A pair of cisterns and a transverse tubule forms a triad. When an AP gets conducted down a TT, calicum is released from the terminal cistern which causes sliding of the filaments. This is called E-C coupling.

13. MUSCLE FIBER TYPES Type 1 Type 2 “Slow Twitch” Small Cell Body
Thinner axon
Type 2
“Fast Twitch”
Larger Cell Body
Thicker Axon
EMG tests type 1, which is recruited first. Therefore steroid myopathy predominately affecting type 2 fibers can’t be tested for.
The central nervous system can increase the strength of muscle contraction by the following:
Increasing the number of active motor units (ie, spatial recruitment)
Increasing the firing rate at which individual motor units fire to optimize the summated tension generated (ie, temporal recruitment)
Both mechanisms occur concurrently. The primary mechanism at lower levels of muscle contraction strength is the addition of more motor units, even though this increases the firing rate of the initially recruited motor units. The recruitment of different units takes precedence over increase in firing rate until nearly all motor units are recruited. At this level and beyond, motor units may be driven to fire in their secondary range to rates greater than 50 Hz.

14. MUSCLE FIBER TYPES

15. NERVE INJURY Seddon’s Classification Neurapraxia (Conduction Block)
Axonotmesis
Neurotmesis
Sunderland’s Classification
Type 1: Neurapraxia
Types 2-4: Axonotmesis
Type 5: Neurotmesis

16. Neurapraxia Demyelination Axon Intact No Wallerian Degeneration
Action Potential slowed
Prognosis Good
This designates a mild degree of neural insult that results in conduction failure across the affected segment. (Conduction Block). It is reversible, and the conducting properties of the nerve above and below the lesion site are NORMAL. Wallerian degeneration does not occur since the axon is intact and there is continuity. It typically occurs from compression and systemic processes that cause local demyelination with AP slowing and failure across the compressed aspect. Large myelinated fibers are more susceptible than small unmyelinated. A mild clinical correlate is the leg falling asleep from crossing our legs.
Fibs should not be observed.

17. Wallerian Degeneration
If an axon is severed, the part of the axon distal to the site of injury will disintegrate. Also known as anterograde degeneration. It occurs at the distal stump of the site of injury and usually begins within 24 hours of a lesion. Prior to degeneration distal axon stumps tend to remain electrically excitable. After injury, the axonal skeleton disintegrates and the axonal membrane breaks apart

18. Axonotmesis Axon disrupted
Connective Tissue (endoneurial/perineural) may or may not be intact.
Wallerian Degeneration DOES occur
Denervation
Prognosis depends
In Seddon, the endoneurium is intact (as are the peri and epi), but degeneration occurs so one can anticipate denervation of the corresponding musculature and absence of sensory modalities. All tissues become inexcitable distal to the site of injury and the length of recovery depends upon the distance from the lesion to the end organ. Prognosis good in Type 2, and gets poorer with Types 3 and 4.

19. Neurotmesis Axon and connective tissue disrupted
Complete severance of nerve
Surgical Repair
Poor prognosis
Poor prognosis even with surgery

20. Mechanisms of Recovery
Remyelination
Collateral Sprouting
Regeneration
Recovery from peripheral nerve trauma may occur by three mechanisms: remyelination, collateral sprouting of axons, and regeneration from the proximal site of injury. Remyelination is the fastest of these reparative processes, occurring over weeks, depending on the extent of the injury. Following degeneration of injured distal axon fragments, collateral sprouts from intact neighboring axons may provide innervation to denervated muscle fibers. This process takes between 2 to 6 months. In cases of severe axonal injury, collateral sprouting is not sufficient to provide innervation to all muscle fibers. Further clinical recovery depends on regeneration from the proximal site of injury, which may require up to 18 months. The timing of recovery depends on the distance of the lesion from the denervated target muscle. Proximal regeneration occurs at a rate of 6--8 mm per day, whereas distal regeneration occurs at 1--2 mm per day [11]. The prerequisite for regeneration is an intact Schwann cell basal lamina tube to guide and support axonal growth to the appropriate target muscle. Schwann cell tubes remain viable for months after injury

21. NERVE INJURY Collateral Sprouting
Recovery from peripheral nerve trauma may occur by three mechanisms: remyelination, collateral sprouting of axons, and regeneration from the proximal site of injury. Remyelination is the fastest of these reparative processes, occurring over weeks, depending on the extent of the injury. Following degeneration of injured distal axon fragments, collateral sprouts from intact neighboring axons may provide innervation to denervated muscle fibers. This process takes between 2 to 6 months. In cases of severe axonal injury, collateral sprouting is not sufficient to provide innervation to all muscle fibers. Further clinical recovery depends on regeneration from the proximal site of injury, which may require up to 18 months. The timing of recovery depends on the distance of the lesion from the denervated target muscle. Proximal regeneration occurs at a rate of 6--8 mm per day, whereas distal regeneration occurs at 1--2 mm per day [11]. The prerequisite for regeneration is an intact Schwann cell basal lamina tube to guide and support axonal growth to the appropriate target muscle. Schwann cell tubes remain viable for months after injury

22. REINNERVATION (FIBER TYPE GROUPING)
In chronic denervating processes such as chronic neuropathy and motor neuron disease, remaining healthy axons sprout and synapse with denervated fibers (collateral reinnervation). As a result of the combined denervation and reinnervation, motor units enlarge, and their fibers, instead of being scattered, come to lie adjacent to one another. In histochemical stains, such motor units appear as groups of myofibers of the same histochemical type (fiber type grouping). When ultimately these motor units lose their innervation and there are no healthy axons left to connect with them, all their fibers shrink together (group atrophy).

23. For Practical Purposes
“Demyelinating Injury”
Neurapraxia (Conduction Block)
Diffuse Demyelination
Good prognosis
“Axonal Injury”
Axonotmesis
Neurotmesis
Prognosis depends on length/severity
Conduction block refers to the inability of an AP to propagate beyond a specific region of nerve.

24. NCS/EMG
NCS
EMG

25. INSTRUMENTATION Electrodes Active (Recording) Reference Ground GROUND
“G1”, “E1”
Action Potential
Electrical “Noise”
Reference
“G2”, “E2”
No Action Potential
Ground
Excess Charge
GROUND G2 G1

26. NERVE CONDUCTION STUDIES
The recorded AP is composed of multiple subcomponent action potentials arising from the individual fibers of the nerve, each with their own slightly different conduction velocities. The fastest fibers will arrive first

27. 3 Main AP measured Compound Motor Action Potential (CMAP)
Sensory Nerve Action Potential (SNAP)
Compound Nerve Action Potential (CNAP)
The concept of 4cm separation does not apply for a CMAP and applies only for sensory studies. Will not go into detail on finer differences among these studies
Summation of action potentials
Amplitude – sum of axons polarized
Latency – fastest fiber

28. NCS Parameters Latency Amplitude Conduction velocity
determined by conduction velocity of the nerve, neuromuscular junction & muscle
Amplitude
determined by number of muscle fibers activated
Conduction velocity
determined by conduction velocity of the fastest fibers

29. Important Patterns Axonal Loss Demyelination Conduction Block
Decreased amplitude
Maintain latency
Demyelination
Prolonged latency
Conduction Block
50% drop in amplitude (variable)
In order to asses for reduced amplitude, comparison to a previous baseline value, control/reference value, or opposite side is needed.
Although axon loss leads to decreased amplitude, the reverse is not always true. (Conduction Block).
Mild slowing of CV and prolongation of latency can occur if largest and fastest fibers are lost. At one extreme, all fibers except the fastest ones can be lost. At the other extreme, all fibers except the slowest fibers can be lost…in which case overall velocity cannot be lower than that of the slowest fibers. The slowest fibers tend to be 75% of the lower limit of normal, thus if overall CV is less then 75% lower limit of normal, this suggests something other than axon loss. For latency, if overall latency is greater than 130% the upper limit of normal, the same applies.

30. Important Patterns Proximal Lesions (Radiculopathy) Sensory Nerve Root
Proximal to DRG
Peripheral Axon Intact
Motor Nerve Root
Distal to Anterior Horn Cell
Axonal Degeneration Occurs

31. NERVE CONDUCTION STUDIES: Important Patterns

32. NEEDLE EMG
Summation of MUAP

33. Needle EMG Parameter Spontaneous Muscle Membrane Electrical Activity
Motor Unit Configuration
Motor Unit Recruitment

34. Spontaneous Activity:
Electrical Waveforms not under voluntary control
Healthy muscle is normally electrically silent at rest
Spontaneous Activity is electrical waveforms that are not under voluntary control. Placing a needle in healthy muscle tissue at rest normally results in complete electrical silence

35. Abnormal Spontaneous Activity
Positive Sharp Wave (“PSW”)
Same significance as Fib
Fibrillation Potential (“Fib”)
Membrane Instability
Denervation
Myopathy
Trauma
In the presence of instability, the muscle’s resting membrane potential becomes less negative (so increases from -80 to -60 mV), and it will oscillate. Thus it is easier for the fiber to reach threshold and depolarize.
Can be triphasic or biphasic. Its regularity typically distinguishes it from an endplate spike
PSWs are typically biphasic with an initial positive phase followed by a slow, small negative return to baseline
Both are regular with a firing frequency from 0.5 to 10 Hz, sometimes up to 30 Hz.

36. NEEDLE EMG: Spontaneous Activity
Pattern recognition

37. Motor Unit Analysis Morphology Stability Recruitment Duration
Amplitude
Phases
Stability
Recruitment
Henaman’s size principle
Duration (measured in ms) is the time from initial deflection from baseline to final return to baseline. It reflects and depends on the number of muscle fibers within the motor unit and the dispersion of their polarizations over time, thus the total depolarization of all the single muscle fibers forming one motor unit. Since all the fibers do not depolarize at the same time, the duration will reflect how dispersed the depolarizations are.
Duration correlates with pitch, so short duration sound crisp and static-like while long duration sound thuddy.
Duration is increased by reinnervation (increased fibers per motor unit) or temporal dispersion. It is decreased by loss of fibers/atrophy.

38. Common Patterns Axonal Loss Demyelinating Lesion Myopathy
PSWs and Fibs present
Decreased Recruitment
MUAP changes
Increased Duration
Polyphasia
Increased Amplitude
Demyelinating Lesion
NO PSW’s and Fibs
No MUAP changes
Myopathy

39. Common Patterns EMG and NCS changes evolve over time…..
Wallerian Degeneration
3-5 days for motor fibers
6-10 days for sensory fibers
Reinnervation

40. Evolution of NCS/EMG Changes
Axonal Loss
<3 days old
No Wallerian Degeneration
DISTAL NCS normal!!
No PSWs/Fibs
Normal MUAPs
Decreased Recruitment
Distal studies will be normal even though the patient may have clinical weakness and sensory loss

41. Evolution of NCS/EMG Changes
Axonal Loss: 1-6 weeks old
NCS:
Decreased Amplitude
Normal CV*
Normal Latency*
EMG:
Fibs/PSWs present
Decreased Recruitment
Normal MUAP
Can have decreased CV and increased latency if fastest fibers are lost
Rule of thumb, hence earliest time for study about 3 weeks

42. Evolution of NCS/EMG Changes
Axonal Loss: Months-Years Later
NCS:
Decreased Amplitude
Normal CV*
Normal Latency*
EMG:
No Fibs/PSWs
Decreased Recruitment
MUAP
Long Duration
High Amplitude
Polyphasic
Amplitude will eventually normalize after years due to regeneration and sprouting. Fibs/PSWs resolve

43. Evolution of NCS/EMG Changes
Demyelination
NCS
Decreased Conduction Velocity
Prolonged Latency
Variable changes in Amplitude
Normal EMG!!
Conduction Block
NCS
Decreased Amplitude
Prolonged Latency
EMG
Decreased Recruitment
Normal MUAPs
No PSWs/Fibs
Both are not affected by time. Conduction Block abnormalities are found only if NCS is performed across the block and if EMG muscle is tested distal to the block.

44. Clinical Use Common Entrapment Syndrome Median at the Wrist (CTS)
Ulnar at the Elbow
Peroneal Palsy at the Fibular Head

45. Carpal Tunnel EDX Grading
Simple Grading Scheme for median neuropathies at the wrist
- Mild --> sensory latencies prolonged
- Moderate --> motor latencies prolonged
- Severe --> motor amplitude reduced and/or evidence of EMG abnormalities on EMG.

46. CTS Clinical Pearl
By 6 months post-surgery, the maximum improvement in latencies will have occurred
1/2 of patients post surgery do not return to normal latencies
Must compare to post-operative latencies to pre-op latenciec to determine an unsuccessful surgery
If no preoperative latencies, wait another 3-6 months and assess the interval change

47. Tarsal Tunnel
EMG: Footwear and trauma cause low level of spontaneous activity in foot muscles
Controversial

48. Radiculopathy
Can confirm the presence of a radiculopathy with or without findings on imaging studies
EMG is not needed in all radic patients
Most useful w/ multi-level pathology on MRI but inconclusive PE
Can help determine location of radiculopathy
Multi-level radics present in 12-30%
Excludes other possible diagnoses
Can determine time course or severity of radiculopathy
1% of lbp, but common request

49. NCS usually WNL in radics; abnormalities are found on needle EMG
SNAP is normal in lesions prox to DRG, and nearly all radics damage nv root proximal to DRG
The NCS is done to r/o other conditions, specifically entrapment neuropathy and plexopathy

50. False positive rates on MRI are 10% (cervical)
Radics can be seen without structural abnormalities on MRI
Sensitivity ranges from 55-84%
Slightly lower compared to MRI
Sensitivity increases w/ neurologic abnormalities
Specificity ranges upto 90-95%
Slightly higher than MRI

51. EMG Caveats Time Course in Radiculopathy
Acute phase: decreased MUAP recruitment but NML morphology
Day 10-14: + waves/fibs in paraspinals
Day 14-21: + waves/fibs in prox peripheral muscles
Day 21-28: + waves/fibs in distal peripheral muscles (up to 5-6 wks total time)
MUAP morphology is the same as denervation occurs, but polyphasia (also paraspinalproxdistal muscles) heralds reinnervation (over the course of months, i.e. chronic radic)

52. Caveats Limitations of Needle EMG May have NML EMG in acute phase
If only demyelination is present, EMG can be NML (only sig CB w/ weakness will give decreased MUAP recruitment (rare in radic))
If sensory root is predominantly affected, EMG will be NML
Different fascicles may be more or less involved (i.e. some muscles of a particular myotome may be involved while others spared)

53. Caveats The “double crush”
Cervical radic (C6-C7) plus median neuropathy at the wrist
C8-T1 radic plus ulnar neuropathy at the elbow
Does not infer that radic predisposes to median neuropathy at the wrist

54. Peripheral Neuropathy

55. Plexopathy Compression (CABG) Inflammatory (Parsonage-Turner Syndrome)
Radiation Injury (Radiotherapy)
Traumatic Injury (Traction, laceration, missile)
Ischemia (Diabetic amyotrophy)

56. Neurogenic Thoracic Outlet Syndrome
Incidence 1:1,000,000
A partial lower trunk plexopathy or C8/T1 root injury
Secondary to prominent C7 transverse process or prominent cervical rib
Normal study focused on brachial plexus trunk essentially rules out

57. Other Pearls
Electrodiagnostic studies are a supplement to, and not a replacement, for the history and physical examination
Electrodiagnostic results are often time-dependent
Electrodiagnostic studies are not “standardized” investigations and may be modified by the practitioner to answer the diagnostic question

58. EDX Testing

59. When to order EDX testing
Neck/arm pain, back/leg pain, suspected CTS, peripheral neuropathy, weakness, wasting, cramps.
Sort out these problems, establish etiology, assess severity, provide objective/prognostic information.
Accurate diagnosis leads to effective treatment.

60. Typical diagnosis for consideration on EDX consultation
Mononeuropathy
Mononeuropathy Multiplex
Radiculopathy
Plexopathy (Brachial or Lumbosacral)
Anterior Horn Cell Disorders
Diffuse neuropathies
Cranial neuropathies
Neuromuscular Junction Disorders
Myopathy
Traumatic nerve injury
Intervention vs wait
Assess improvement
18 month time frame
Or to help rule out, not fishing
Need to have some clinical suspicion

61. When Not to order EDX
Central Nervous System Disorders (Stroke, TIA, Encephalopathy, spinal cord injury)
Multiple Sclerosis
Total body fatigue, fibromyalgia
Joint pain
EDX consult is not a substitute for PM&R/Neurology/Orthopedic etc…

62. Counseling Patients
Inform the patient about the test and the reasons behind it.
Give them heads up about what to expect.
Small gauge solid needle test portion
Electrical stimulation portion
Duration of test depends on findings but typically about 60minutes.
Not the most comfortable but tolerable for just about anyone.
Risks very small. Verbal consent only.

63. Reading EMG Reports Tailored to referring provider
Specific questions from ie Hand surgeon, spine surgeon
Fuzzier question, ie generalized weakness
Two broad styles
Tabular or narrative
Most read final impression only
Clinical Management usually deferred to referring provider
Clinical vs Electro diagnostic impression
An outline of the localization, severity, and acuity of the process
Notation of other diagnoses that are detected/excluded
Explanation of any technical problems

64. Summary: Utility of EMG/NCS
Highly sensitive indicator of early nerve injury
Detects dynamic and functional injury missed by MRI
Provides information regarding chronicity of nerve injury
Provides prognostic data
Highly localizing
Clarifies clinical scenarios when one disorder mimics another
Identifies combined multi-site injury, avoiding missed diagnoses
Identifies more global neuromuscular injury with focal onset
Provides longitudinal data for charting course, response to therapy

65. QUESTIONS ?