C H A P T E R
18
Needle EMG Abnormalities in
Neurogenic and Muscle Diseases
K. Ming Chan
TYPES OF INTRAMUSCULAR NEEDLE EMG
ELECTRODES
RATIONALE FOR THE CHOICE OF ELECTRODES
NORMAL FINDINGS IN HEALTHY SUBJECTS
TYPICAL PATHOLOGICAL NEEDLE EMG FINDINGS
IN PATIENTS WITH AXON LOSS NEUROGENIC
DISEASES
DEMYELINATING NEUROGENIC DISEASES
UPPER MOTONEURON DISEASES
PRIMARY MUSCLE DISEASES
POTENTIAL PITFALLS IN DISTINGUISHING
NEUROGENIC FROM MUSCLE DISEASES BASED
ON THE NEEDLE EMG FINDINGS
Muscle Selection
The physiological properties of motor units can
be affected in many different ways, depending on
the underlying disease process. Recognition and an
understanding of these patterns of abnormalities
can be helpful when one tries to determine the
mechanisms of injury and to quantify disease sever-
ity. The common abnormal ndings in pathological
conditions can be broadly divided as those associ-
ated with neurogenic versus those associated with
myopathic diseases. This approach is useful concep-
tually in illustrating how motor unit physiological
functions are altered, depending on the location and
nature of the primary pathology. However, along
with this generalization comes the risk of oversim-
plication. There are often exceptions to these rules
and many abnormalities are not unique to either
neurogenic or muscle diseases. To avoid these pit-
falls, an understanding of the characteristics of the
different types of needle electrodes and an apprecia-
tion of the range of normality and factors that can
affect them are necessary.
In this chapter, the following topics are covered:
(1) different types of needle electromyographic
(EMG) electrodes, (2) rationale for their choice, (3)
needle EMG ndings in normal individuals to help
contrast differences in (4) pathological conditions,
and nally (5) potential technical and physiological
pitfalls in the interpretation of needle EMG abnor-
malities.
TYPES OF INTRAMUSCULAR
NEEDLE EMG ELECTRODES
The study of many motor unit electrophysiologi-
cal properties, such as the size of the motor unit
Adequate Motor Unit Sampling
Time Course of the Disease
Temperature
Choice of Recording Needle Electrodes and Their
Site of Insertion
Aging
Patient Cooperation
Fatigue and Other Physiological Factors
action potential, the ring rate, synchronicity of the
electrical conduction, security of electrical transmis-
sion through the terminal branches and neuromus-
cular junction, and excitability of muscle ber mem-
brane, axon, and motoneuron require the use of
microelectrodes that can be placed close to the
innervated muscle bers. Many types of intramuscu-
lar needle electrodes have been specically de-
signed to examine different physiological parame-
ters (Fig. 181). To make a sensible choice of the
type of electrodes that can best measure the physio-
logical function of interest, a clinician needs to have
a good understanding of the specic features associ-
ated with each electrode type, their limitations, cost,
availability, and potential risk.
The commonly used concentric needle electrode,
introduced by Adrian and Bronk in the 1920s, has a
single insulated wire inside the cannula of a hypo-
dermic needle, xed in place by epoxy glue and cut
ush with the needle tip (Adrian and Bronk, 1929).
This recording wire, with a recording surface of 150
by 600 m at the tip, is referenced to the cannula.
Another commonly used electrode is a monopolar
needle electrode that is made up of an insulated
solid needle except at the most distal 300 m at the
tip, referenced to a surface electrode; thus, it has a
slightly larger pickup area.
To study electrical transmission in single muscle
bers, an electrode with a much smaller recording
area is required. This electrode, introduced by Stal-
berg and Ekstedt in the 1960s, with a recording
surface of 25 m, is located in a side port 3 mm
back from the needle tip on the opposite side of the
bevel. (Ekstedt, 1964; Stalberg, 1966). This congu-
359
360 Section IV Peripheral Motor Sensation
Figure 181. Four common types of needle EMG electrodes. 1,
Concentric needle electrode. The recording wire is represented
by the stippled area, running the length of a hypodermic needle.
A full view of the recording surface is shown on the right. 2, A
monopolar needle electrode that is simply a wire insulated all
around except at the tip. 3, Single-ber electrode. 4, Macro-EMG
electrode. The setup on the right side (4B) is identical to that of
the single-ber electrode. At the tip of the needle is a very large
recording surface (the area in black measuring 1.5 cm in length)
for detecting the action potentials generated by all the constit-
uent muscle bers within the motor unit territory (A). A full
description of these electrodes is in the text. (Adapted by permis-
sion from Stalberg E: Single ber EMG, macro EMG, and scanning
EMG: New ways of looking at the motor unit. CRC Crit Rev Clin
Neurobiol 1986;2:127.)
ration helps to minimize the risk of studying muscle
bers damaged by the needle tip during insertion.
Diameter of the uptake area is about 300 m.
Given that the innervation territory of a motor
unit can be up to 1 cm in normal individuals and
even larger in pathological conditions, an electrode
with a pickup territory larger than those described
so far is obviously needed. The macro-EMG elec-
trode was introduced by Stalberg for this purpose
(Stalberg, 1980). The conguration of this electrode
is similar to the single ber electrode except that
the distal 1.5 cm of the needle electrode is bare.
This recording surface, referenced to a surface elec-
trode on the skin, has a very large pickup area and
can therefore detect the entire motor unit territory;
however, to record from the entire territory of the
motor unit, a two-channel setup is required. The rst
channel uses the action potential spike generated by
a single muscle ber, lying within the pickup area of
the single ber electrode, as a trigger. The action
potentials from all other muscle bers innervated
by the same motoneuron, time locked to this spike,
are averaged and displayed on the second channel.
RATIONALE FOR THE CHOICE OF
ELECTRODES
Depending on the particular physiological proper-
ties of interest, the clinician has to choose an elec-
trode that is most appropriate for the task at hand.
Certain physiological properties are best measured
by electrodes with a highly restricted pickup area,
while the opposite may be true for others.
For studying neuromuscular transmission and
muscle ber density, the single-ber EMG electrode
is ideal, as action potentials generated by individual
muscle bers lying within the very small pickup area
of the electrode can be readily discerned and the
jitter between the ber pairs measured.
On the other hand, the small pickup area of the
electrode does not provide any information on the
electrical size of the whole motor unit. Although this
information may be obtained with the use of surface
electrodes for supercial muscles, a macro-EMG nee-
dle electrode is needed for deeper muscles (Stal-
berg, 1966; Barkhaus and Nandedkar, 1994).
The study of recruitment threshold and ring rate
of individual motor units requires reliable identi-
cation of their motor unit action potentials. There-
fore, it is crucial that the conguration of the action
potentials of the recruited motor units has to remain
relatively constant even with small needle displace-
ment. For these purposes, monopolar and concen-
tric needles are reasonable choices.
In addition to the aforementioned considerations,
the risk of infection, baseline interference, electrical
noise, and patient comfort should also be taken into
account. With increasing concern regarding blood-
borne diseases, such as human immunodeciency
virus (HIV) and hepatitis, disposable needle elec-
trodes are used at an increasingly frequently rate.
An added drawback of reusable electrodes is that
regular maintenance is required. The recording sur-
face of the electrode must be kept meticulously
clean in order to minimize impedance and, hence,
baseline noise. The insulation coating of the elec-
trode also has to be regularly inspected, as chipping
can also degrade the signal-to-noise ratio. Regular
sharpening and inspection for hooks at the tip are
also necessary in order to minimize patient discom-
fort and damage to the muscle bers.
NORMAL FINDINGS IN HEALTHY
SUBJECTS
The rst electrical signal one sees on needle EMG
examination is insertional activity generated by the
muscle bers when they come into contact with the
needle. In normal muscles, this only lasts 50 to 150
ms. An exception is when the needle is placed at
the motor end-plate where irregular discharges will
be detected (Fig. 182). These include miniature
end-plate potentials (MEPPs) and end-plate poten-
tials (EPPs).
A MEPP is the result of depolarization of the post-
synaptic membrane generated by the binding of an
acetylcholine vesicle to an acetylcholine receptor.
The MEPPs are irregular, small-amplitude, monopha-
sic high-frequency discharges with a characteristic
seashell sound. These discharges are nonpropa-
gating membrane potentials occurring spontane-
ously even when the subject is at rest. With motor
unit recruitment and subsequent generation of an
action potential in the terminal axon, the frequency
and number of the MEPPs will increase, resulting in
spatial and temporal summation to generate an EPP
with sufcient amplitude to cause opening of the
 Chapter 18 Needle EMG Abnormalities in Neurogenic and Muscle Diseases 361

mal spontaneous or insertional activity. Two clues

Figure 182. Recordings obtained by inserting a concentric nee-
dle electrode into the end-plate region of a tibialis anterior mus-
cle. Miniature end-plate potentials (MEPPs) can be seen ring at
high frequency immediately after needle insertion (A). The activ-
ity died down quickly within one (B) to two minutes (C) when
only a few MEPPs are seen. (Reprinted by permission from Brown
WF: The Physiological and Technical Basis of Electromyography.
Boston, Butterworth, 1984;373.)
voltage-sensitive Na channels. This results in the
generation of a propagating action potential that will
go on to depolarize the rest of the muscle ber. Brief
trains of EPP may also arise owing to irritation by
the advancing needle.
In routine needle EMG examination, the examiner
usually inserts the needle close to the motor end-
plate, where the rising slope of the motor unit action
potential is sharper. Although one tries to avoid
entering the motor end-plate region because of the
associated discomfort, it may be encountered inad-
vertently, particularly in small muscles such as
those in the hands, feet, or paraspinal muscles. The
end-plate activity could be easily mistaken as abnor-
that help the examiner to recognize the motor end-
plate zone are that (1) the EPPs are small and brief
in duration. Being generated at the end-plate, they
do not have a preceding positive deection and are
therefore biphasic in conguration; and (2) the
MEPPs persist even with the subject completely re-
laxed but quickly disappear with a small movement
of the needle electrode away from the motor end-
plate.
Normal motor units are typically recruited at 6 to
7 Hz, ring semi-irregularly. With increasing ring
frequency, the force generated increases in a sigmoi-
dal fashion, rather than linearly. Depending on the
contractile speed of the motor units, the steepest
incline usually lies between 15 and 30 Hz. Although
motor units sometimes re very rapidly at the begin-
ning of a ramp contraction, they rarely re at rates
higher than 30 Hz once the force has reached a
stable plateau during an isometric contraction. In
addition to maintaining the force of contraction
through modulation of ring rate, recruitment of
additional motor units is another means of increas-
ing force production. One way of gauging this is
by calculating the recruitment frequencythe ring
rate of the recruited motor unit when an additional
motor unit is recruited. Recruitment frequency in
normal individuals has a range between 6 and 15
Hz. Alternatively, the recruitment ratio can be used:
dividing the ring frequency of the fastest ring
motor unit by the number of different motor units
detected by the needle electrode.
Normal motor unit action potentials are typically
triphasic in their conguration: an initial small, posi-
tive deection followed by a larger negative peak
and a subsequent slowly recovering positive phase
(Fig. 183A). The constituent muscle bers even in

Figure 183. Typical motor unit congurations in myopathic and neuropathic diseases. B, In contrast to a normal motor unit (A), a
myopathic motor unit is smaller, shorter with polyphasicity. However, many motor units also have linked potentialsa result of muscle
ber splitting, regeneration of the terminal axons, or changes in the caliber of muscle bers. C, On the other hand, a neuropathic
motor unit undergoing recent reinnervation has an increased duration, number of phases, and linked potentials. As the motor unit
matures, the motor unit amplitude will gradually increase and the polyphasicity will be reduced. (Reprinted by permission from Brown
WF: The Physiological and Technical Basis of Electromyography. Boston, Butterworth, 1984;318.)
362 Section IV Peripheral Motor Sensation

a normal motor unit do not re completely synchro-

nously. This variability is accounted for by the vari-
ance in temporal summation and postsynaptic depo-
larization and is best appreciated with a single-ber
EMG needle when the jitter can be clearly seen. In
normal muscles, the jitter is about 10 to 30 s.
TYPICAL PATHOLOGICAL NEEDLE
EMG FINDINGS IN PATIENTS WITH
AXON LOSS NEUROGENIC
Table 181. A Clinical Scale for Grading the Fibrillation
Potentials and Positive Sharp Waves
0 Normal insertional activity
1 Transient but reproducible brillation potentials and/or
positive sharp waves with needle movements
2 Occasional spontaneous activities in more than two sites
3 Moderate spontaneous activities in all needle sites
4 Abundant spontaneous activities lling the screen
From Miller RG, Peterson GW, Daube JR, Albers JW: Prognostic value of
electrodiagnosis in Guillain-Barre syndrome. Muscle Nerve 1988; 11:769
774.

DISEASES
Denervated muscle bers become hyperexcitable,
reected by the presence of brillation potentials
and positive sharp waves either ring spontane-
ously or induced by needle movements. Fibrillation
potentials are small biphasic or triphasic muscle
ber action potentials with brief duration, while pos-
itive sharp waves have a large steep initial positivity
followed by a slow recovering negative phase (Fig.
184). While there is convincing evidence that bril-
lation potentials are extracellularly recorded action
potentials generated by single muscle bers, the
origin of positive sharp waves is much less clear
(Dumitru, 1996). Both positive sharp waves and -
brillation potentials most commonly discharge regu-
larly with the sound likened to the ticking of a
clock. This hyperexcitability was initially thought
to be due to hypersensitivity of muscle bers to
acetylcholine following denervation. However, this
did not turn out to be the case. Rather, they may be
due to altered Na channel density and kinetics, lead-
ing to partial depolarization and spontaneous oscil-
lation of the membrane potential (Thesleff and
Wand, 1975). They gradually disappear, however, as
reinnervation progresses. Clinically, these changes
are commonly represented on a ve-point scale (Ta-
ble 181) (Miller et al., 1988). Although simple and
easy to use, this scale is nonlinear and qualitative,
which limits its usefulness. Hyperexcitability of pe-
ripheral nerve bers can also be expressed by other
abnormalities. Myokymic discharges characteristi-
cally consist of bursts of potentials interspersed
with periods of electrical silence, with a sound lik-
ened to that of marching soldiers. The number
and frequency of discharges of individual potentials
in the burst and the burst duration and frequency
can be quite variable (Albers et al., 1981). The con-
guration of the action potentials suggests that they
are generated by a part of or an entire motor unit.
The precise origins of myokymic discharges are un-
known. They probably represent ectopic spontane-
ous discharges generated by injured and com-
pressed nerve bers. Common conditions in which
myokymic discharges are found are summarized in
Table 182.
Fasciculation is another consequence of periph-
eral nerve hyperexcitability. Fasciculation potentials
discharge irregularly at rates as low as 0.1 Hz, up to
several hertz (Fig. 185). The associated sound of
this irregularly discharging pattern has been likened
to the sound of raindrops on a tin roof. The sites
at which the fasciculation potentials originate can
be anywhere from the dendritic tree to the terminal
arbor of the lower motoneuron, accounting for the
variable morphology of the action potentials (Con-
radi et al., 1982; Roth, 1982). Although fasciculation
potentials are particularly common and well known
in certain diseases, such as amyotrophic lateral scle-
rosis, they can also occur in normal individuals.
Although some earlier studies suggested that there
might be reliable distinguishing features between
fasciculation potentials in pathological and normal
states, this did not turn out to be so (Trojaborg and
Buchthal, 1965).

Figure 184. Recording from the

tibialis anterior muscle of an 81-
year-old woman with a peroneal
nerve injury using a concentric
needle electrode when the pa-
tient was at rest. Most of the ab-
normal spontaneous activities
on the right panel are positive
sharp waves with a regular ring
pattern at about 30 Hz. A bril-
lation potential is also present,
ring regularly at a much lower
frequency. The action potentials
on the left panel have a brilla-
tion potential ring regularly at
12 Hz.
 Chapter 18 Needle EMG Abnormalities in Neurogenic and Muscle Diseases 363

Table 182. Diseases Associated with

Myokymic Discharges

Central Nervous System Diseases

Multiple sclerosis
Pontine tumors
Facial nerve palsy
Spinal cord injury
Peripheral Nervous System Diseases
Radiation plexopathy
Facial nerve palsy
Guillain-Barre syndrome
Vasculitic and ischemia neuropathies

Figure 186. Recording using a monopolar electrode from the

In an axonal injury process, apart from changes
in the recruitment pattern, conguration of the mo-
tor unit action potential is also changed. In early
stages of reinnervation, during the initial weeks and
months, newly reinnervated motor units are small,
with many spike components and sometimes linked
potentials (Fig. 186). The late components are gen-
erated by newly formed terminal twigs that are
thinly myelinated and therefore can only conduct
slowly. Furthermore, electrical propagation is some-
times insecure, especially at high ring frequency.
Consequently, conduction block and a dropout of
these components may occur. This instability is par-
ticularly evident when it is studied with a single-
ber EMG needle. Markedly increased jitter and fre-
quency-dependent blocks of the late components
can be frequently seen (Stalberg and Trontelj, 1994).
As the caliber of these terminal branches be-
comes larger and better myelinated, electrical con-
duction will be faster and more secure, resulting in
disappearance of the polyphasic components and
linked potentialseventually replaced by a triphasic
but enlarged motor unit action potential (see Fig.
183C).
With the reduced number of motor units and less
intermingling of muscle bers belonging to different
motor units in the same area, fewer motor units are
detected by the recording needle electrode. As a
result, the interference pattern becomes discrete
and incomplete even with maximal effort. However,
the ring rate of the individual motor unit is in-
creased, as there are fewer motor units contributing
to the force production (Fig. 187).
biceps brachii muscle of a 59-year-old man with a very severe
axon loss sensorimotor polyneuropathy. It shows a hypercomplex
motor unit action potential with a very small link potential (*).
Instability of some of the spike components is evident from the
variation in the conguration of the motor unit action potentials.
DEMYELINATING NEUROGENIC
DISEASES
A primary consequence of this is conduction
block, resulting in fewer available motor units for
voluntary recruitment. Thus, the interference pat-
tern becomes discrete, but the ring rate of the
recruitable motor units may be increased. Occasion-
ally, recurrent discharge of the same motor units in
the form of doublets or triplets can be seen as a
result of ephaptic transmission or recurrent activa-
tion of the same motor axons. If there is additional
secondary axon loss, then increased insertional ac-
tivity, brillation potentials, and positive sharp
waves will also appear. In an experimental demyelin-
ation model in rats, Sumner and colleagues showed
that the smaller, lower threshold motor units were
more susceptible to conduction block (Sumner et
al., 1982). As a result, the remaining motor units
may have larger-than-expected amplitudes.
UPPER MOTONEURON DISEASES
Characteristically, the most notable abnormality
in patients with upper motoneuron diseases is a
decit in voluntary recruitment. Motor units, even

Figure 185. Fasciculation po-

tentials. This record shows the
characteristic highly irregular
ring pattern of fasciculation po-
tentials with low ring rates.
Judging from the differences in
their amplitudes, there are many
fasciculating motor units. (Re-
printed by permission from
Brown WF: The Physiological
and Technical Basis of Electro-
myography. Boston, Butter-
worth, 1984;345.)
364 Section IV Peripheral Motor Sensation
Figure 187. A monopolar needle recording from the tibialis ante-
rior muscle of a 60-year-old man who sustained a severe sciatic
nerve injury after a hip dislocation 2 years earlier with the use
of a monopolar electrode. In this record, a lone motor unit was
recruited, ring at frequencies between 15 and 25 Hz without any
sign of other additional motor unit recruitment.
when recruited, can re only slowly and in a poorly
sustained manner.
In addition to this, however, there are other fea-
tures. Although less appreciated, increased inser-
tional activity, spontaneous discharge of brillation
potentials, and positive sharp waves can follow
within several weeks after an upper motoneuron
lesion in the muscles on the contralateral side, par-
ticularly in the distal limb. The underlying mecha-
nism is thought to be due to transsynaptic degener-
ation, when the loss of input from the upper
motoneurons induces death of the lower motoneu-
rons. This phenomenon is well documented in hu-
mans (Goldby, 1957; Goldkamp, 1967; McComas et
al., 1973; Brown and Snow, 1990) monkeys (Mat-
thews et al., 1960), and other mammalian species
(Cook et al., 1951). As well, myokymic discharges
can accompany central nervous system disorders,
Figure 188. Three recordings from the tibialis anterior muscle of the same patient in Figure 184 while the patient was at rest. These
regularly ring action potentials are complex repetitive discharges brought on by needle movements. Congurations of the action
potentials in the three panels are markedly different, depending on the action potentials generated by particular muscle bers involved
in the closed circuit, activated and sustained through ephaptic transmission.
such as multiple sclerosis, other brainstem diseases,
and spinal cord injury.
PRIMARY MUSCLE DISEASES
Increased insertional activity or abnormal sponta-
neous activity may be present if there has been a
substantial amount of muscle ber necrosis. The
fact that increased insertional activity is also pres-
ent in muscle diseases, such as polymyositis, can
be explained by segmental muscle ber necrosis,
which effectively results in denervation of the sur-
viving portion of the muscle bers. When the muscle
ber membrane excitability is increased further,
these electrical activities may become spontaneous.
Although abnormally increased insertional activ-
ity is a well-known abnormality in a denervation
process or with muscle ber necrosis, reduced in-
sertional activity is also abnormal. This may occur
in a number of settings, including muscle brosis,
fatty inltrates, and electrolyte imbalance, such as
profound hypokalemia or during periodic paralysis.
An important clue to muscle ber replacement by
brosis or fatty tissue is that the consistency of the
muscle and resistance to the advancing needle are
changed. In the case of brotic tissues, the muscle
feels rubbery and its resistance to needle insertion
is increased. Conversely, in the case of fatty inl-
trate, resistance is reduced.
In more chronic processes, complex repetitive dis-
charges (CRDs) may also be present. The complex
conguration of CRDs is explained by the fact that
they consist of action potentials generated by indi-
vidual muscle bers forming a closed circuit, acti-
vated through ephaptic transmission (Fig. 188)
(Trontelj and Stalberg, 1983). The initially activated
muscle ber acts as a pacemaker, initiating the ring
at a fairly constant rate until the membrane poten-
tial eventually runs down to the point when ring
 Chapter 18
Table 183. Diseases Associated with Complex
Repetitive Discharges
Neurogenic Diseases
Spinal muscular atrophy
Charcot-Marie-Tooth disease
Amyotrophic lateral sclerosis
Radiculopathy
Chronic polyneuropathies
Primary Muscle Diseases
Inammatory myositis
Muscular dystrophies, particularly in
Duchennes muscular dystrophy
stops abruptly. CRDs are present not only in primary
muscle diseases but also in many chronic axon loss
neurogenic disorders. The more commonly associ-
ated conditions are listed in Table 183.
In myotonic disorders, muscle bers may be ab-
normally excitable, resulting in muscle stiffness.
Classically, on needle examination, myotonic dis-
charges have a cyclical waxing and waning ring
pattern with characteristic sounds likened to those
coming from a revving motorcycle or a dive-
bomber (Fig. 189). The morphological appearance
of the action potentials, brief biphasic or sometimes
monophasic spikes, suggests that they are probably
generated by single muscle bers. Clinically, this
symptom is particularly troublesome in myotonia
congenita, more so than in myotonic dystrophy.
However, the muscle stiffness tends to improve
when the muscle warms up. Patients with paramyo-
tonia are particularly sensitive to cold when severe
muscle contracture can develop. Myotonic dis-
charges can have a number of underlying mecha-
nisms including dysfunctional changes affecting the
ion channels (Ptacek et al., 1993). The end result is
that the resting membrane potential becomes unsta-
ble, at times partially depolarized and hence hyper-
excitable (Iaizzo, 1991). Although similar in some
ways, neuromyotonia should not be confused with
myotonia. Neuromyotonic discharges can have a
wide variety of ring patterns, sometimes at rates
as high as several hundred hertz (Fig. 1810). This
is associated with rare conditions such as Isaacs
Figure 189. Myotonic discharges with a characteristic waxing
and waning pattern, in both amplitude and frequency, giving rise
to the classic motorcycle or dive-bomber sound. (Reprinted
by permission from Kimura J: Electrodiagnosis in Diseases of
Nerve and Muscle: Principles and Practice, 2nd ed. New York,
Oxford University Press, 657. Copyright 1989 by Oxford University
Press, Inc.)
Needle EMG Abnormalities in Neurogenic and Muscle Diseases 365
syndrome or stiff-man syndrome (Newsom-Davis
and Mills, 1993).
The motor unit action potential conguration in
primary muscle diseases is also altered. As the re-
sult of muscle ber atrophy, the motor unit ampli-
tude is reduced. However, this is not always ob-
served in recordings with concentric and monopolar
electrodes as the motor unit action potential ampli-
tude is highly inuenced by the muscle bers lo-
cated immediately adjacent to the recording surface
of these electrodes. In contrast, the duration of the
motor unit action potential, which is typically re-
duced in muscle diseases, has been found to be a
more reliable parameter (Kugelberg, 1949; Buchthal
and Pinelli, 1953). The reduced duration is thought
to be the result of muscle ber loss resulting in
less temporal dispersion. However, an observation
against this is that as the affected muscle bers
undergo varying degrees of atrophy, their conduc-
tion velocities also become increasingly varied
which, in turn, would lengthen the duration. This
fact could explain the high incidence of late compo-
nents found in patients with Duchenne muscular
dystrophy (Desmedt and Borenstein, 1976).
The interference pattern is usually full very early
even at very low levels of contraction because of
the limited force-generating capacity of the affected
muscle bers.
POTENTIAL PITFALLS IN
DISTINGUISHING NEUROGENIC
FROM MUSCLE DISEASES BASED
ON THE NEEDLE EMG FINDINGS
Although there are many differences in needle
EMG examination ndings that can help to distin-
guish neurogenic disorders from muscle diseases,
many abnormalities are not unique to either entity.
For example, brillation potentials, positive sharp
waves, and CRDs can be seen in both. The same is
true for small amplitude, hypercomplex motor unit
action potentials. Even though large-amplitude mo-
tor unit action potentials are classically associated
with axon loss neuropathies, the amplitude may pro-
gressively diminish as the disease progresses when
the terminal branches begin to die off and muscle
atrophy ensues. The opposite may be seen in se-
verely affected muscles in muscle diseases when
secondary axonal degeneration can occur, giving
rise to large motor unit action potentials. Therefore,
clinical information is crucial in guiding the proper
interpretation of the EMG ndings.
In addition, a number of potential confounding
factors may further blur the distinction between the
two entities:
Muscle Selection
Different muscles are often preferentially affected
in different diseases. To optimize the sensitivity of
366 Section IV Peripheral Motor Sensation

Figure 1810. Monopolar needle

recordings from a 69-year-old
woman with Isaacs syndrome
while the patient was at rest.
Neuromyotonic discharges were
present in numerous muscles on
her arms, legs, trunk, and face,
of which the aforementioned
four muscles are just a few ex-
amples. The spontaneous ring
did not disappear even when the
patient was sedated and during
sleep, one feature that differenti-
ated it from stiff-man syndrome.
The discharges are mostly of
such high frequencies that it is
impossible to discern the con-
guration of the individual ac-
tion potentials.

the needle EMG examination, appropriate muscle malities evolve over time. For example, following an
selection is crucial. For example, most myopathies acute axon loss injury, brillation potentials and
affect proximal muscles more severely where the positive sharp waves could take up to several weeks
examination should be directed. Examination of to develop, depending on the distance between the
moderately weak muscles is often more helpful than site of injury and the muscle studied. In early stages
looking at muscles that are already markedly af- of a nerve injury, the brillation potentials and posi-
fected, as the pathological features can become tive sharp waves rst appear in muscles immedi-
murky in advanced disease. Distal muscles inner- ately downstream from the site of injury and later in
vated by nerves, such as the median or ulnar the more distal muscles. For example, in a cervical
nerves, that are prone to focal compression may radiculopathy, it may take up to 3 weeks before
add confounding features related to the compres- these changes are seen in the distal forearm and
sion, rather than the primary disease of interest. hand muscles. Over time, these abnormalities even-
tually disappear as the denervated muscle bers
Adequate Motor Unit Sampling
As well, an adequate number of sites in the muscle
must be studied as the affected areas may be widely
scattered, as is often the case in mild polymyositis.
The same also applies to neurogenic diseases, such
as a radiculopathy, in which the pathological
changes may be found only in a few areas in some
of the innervated muscles. Since the recording sur-
face of most needle EMG electrodes is highly re-
stricted, the number of motor units that can be
sampled at any one site is limited. This limitation is
further compounded by the facts that the physiolog-
ical properties of the constituent motor units in a
muscle usually span a wide range and that there is
often a considerable overlap in their distribution
between normal and disease (Fig. 1811). This fur-
ther emphasizes the need for adequate sampling
at different sites as a great necessity. Conversely,
interpretation based on isolated ndings of one or
two large or polyphasic motor unit action potentials
can be potentially misleading. Figure 1811. The distributions of motor unit action potential
duration in patients with polymyositis compared with normal
subjects. The data are superimposed to illustrate the substantial
Time Course of the Disease overlap between the two groups. Therefore, adequate sampling
is crucial to avoiding misinterpretation. (Adapted by permission
Knowledge of this and the rate of progression are from Buchthal F, Pinelli P: Muscle action potentials in polymyo-
also important as most electrophysiological abnor- sitis. Neurology 1953;3:429.)
 Chapter 18 Needle EMG Abnormalities in Neurogenic and Muscle Diseases 367
become reinnervated and replaced by small, hyper-
complex motor unit action potentials. However, if
there is ongoing denervation, the positive sharp
waves and brillation potentials may continue to
persist indenitely (Cashman et al., 1987).
Temperature
Temperature can have a profound inuence on
neuromuscular transmission and propagation of the
action potential along the muscle bers. Cold tem-
perature increases the safety margin of neuromuscu-
lar junction transmission by reducing the release of
acetylcholine vesicles from the presynaptic termi-
nal, decreasing the rate of degradation of the acetyl-
choline molecules in the synaptic cleft, and increas-
ing the sensitivity of the acetylcholine receptors on
the postsynaptic membrane. As a result, the number
and frequency of MEPPs are reduced, and the jitter
is increased. It also increases the time constant of
Na and K channel opening, resulting in a marked
lengthening of the muscle ber action potential du-
ration. As a result of conduction slowing, the num-
ber of spikes in a motor unit action potential in-
creases, resulting in polyphasicity. The amplitude
falls moderately with cooling, presumably as the
result of increased phase cancellation (Buchthal et
al., 1954). Spontaneous activity, a reection of mem-
brane stability, is reduced with cooling.
Choice of Recording Needle
Electrodes and Their Site of
Insertion
Another important physical factor is the relative
size of the pickup area of the electrode used in
comparison to the size of the muscle. A major mis-
match, such as when a macro-EMG needle with a
large pickup area is used to record from a small
intrinsic or foot muscle, could result in a very noisy
baseline from the ring of neighboring muscles. The
site of needle insertion in relation to the motor end-
plate and the angle of insertion in relation to the
muscle ber plane can also affect the conguration
and duration of the motor unit action potential. Fi-
nally, the amplitude of a motor unit action potential
recorded by concentric or monopolar needle elec-
trode is highly inuenced by the muscle bers in
the immediate vicinity of the recording surface. This
is particularly likely to occur in conditions in which
the ber density is increased, as is often the case
in many muscle diseases. Therefore, the amplitude
measured by a macro-EMG needle electrode more
accurately reects the overall size of the motor unit.
Finally, compared with bipolar electrodes, duration
of the motor unit action potential is longer when
recorded with concentric and monopolar electrodes.
Aging
Motor unit loss associated with aging is well
known (Campbell et al., 1973; Doherty et al., 1993;
Larsson and Ansved, 1995). Since the rate of loss is
usually very slow, no abnormal insertional or spon-
taneous activity is detected in the healthy elderly.
However, as the result of chronic reinnervation, the
amplitude and duration of the motor unit action
potentials are increased. In the elderly, the rate and
extent of loss may be more rapid and more marked
in muscles that are prone to trauma or innervated
by nerves vulnerable to compression. Such exam-
ples include the intrinsic hand muscles and the ex-
tensor digitorum brevis muscle in the foot. In these
muscles, a larger proportion of enlarged motor units
may be present.
Patient Cooperation
The ability to volitionally recruit motor units is
obviously inuenced by the subjects cooperation.
Poor recruitment could be the result of a subjects
unwillingness to cooperate, inability to follow com-
mands, misunderstanding of the required task, or
pain. However, despite these difculties, the needle
examination is still potentially useful as the motor
unit recruitment frequency and recruitment ratio are
not inuenced by the previously mentioned voli-
tional factors.
Fatigue and Other Physiological
Factors
Muscle fatigue may occur after prolonged contrac-
tions, sometimes required when one attempts to
record a large number of motor unit action poten-
tials for quantitative analysis. Fatigue can induce
changes in the conguration of the motor unit action
potentials and alter their recruitment pattern and
ring rate (Maton, 1981; Bigland-Ritchie et al., 1986;
Dorfman et al., 1990; Miller et al., 1996; Christova
and Kossev, 1998; Grifn et al., 1998). Hyperventila-
tion and limb ischemia could lead to fasciculation
potentials in otherwise healthy individuals.
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