SECTION VII • Electromyography in Special Clinical Settings
Approach to Pediatric
Electromyography
In conjunction with the clinical examination, electrodiag-
nostic (EDX) studies frequently play a key role in the
evaluation of neuromuscular disorders in infants and chil-
dren. Indeed, there are a large number of neuromuscular
disorders that present in the pediatric age group. In many
of these cases, EDX studies are used to help guide further
evaluation (e.g., muscle biopsy, genetic testing); less com-
monly, they can make a definitive diagnosis. A complete
discussion of pediatric neuromuscular disorders and elec-
trodiagnosis is beyond the scope and purpose of this chapter
(see Suggested Readings). Although the fundamental prin-
ciples of EDX studies are the same for pediatric and adult
age groups, there are significant differences that the elec-
tromyographer needs to keep in mind when studying
infants and children. These differences include both physi-
ologic and non-physiologic factors that may vary consider-
ably between age groups.
NEUROMUSCULAR DIAGNOSES
ARE DIFFERENT IN CHILDREN
THAN IN ADULTS
The most common referral diagnoses to the typical elec-
tromyography (EMG) laboratory include radiculopathy,
polyneuropathy, and carpal tunnel syndrome. However,
adults are more commonly studied in the EMG laboratory,
so this group of diagnoses reflects neuromuscular condi-
tions seen in the adult age group. In contrast, the neu-
romuscular disorders seen in children often are different.
For example, entrapment neuropathies are very common
in adults but are extremely rare in children. Likewise,
radiculopathy, probably the most common of all EMG
referral diagnoses, is virtually unheard of in children,
except in cases of trauma. Although peripheral neuropa-
thies occur in children, they are most often genetic,
whereas most peripheral neuropathies in adults referred to
the EMG laboratory are acquired disorders, usually toxic,
metabolic, inflammatory, or associated with other coexist-
ent medical illnesses. Unlike adults, the more common
diagnoses in children referred to the EMG laboratory are
inherited disorders of the motor unit, including the ante-
rior horn cell (e.g., spinal muscular atrophy), peripheral
nerve (e.g., Charcot–Marie–Tooth), or muscle (e.g., mus-
cular dystrophy).
©2013 Elsevier Inc
DOI: 10.1016/B978-1-4557-2672-1.00038-6
Downloaded from ClinicalKey.com at Univ Targu Mures Med Pharmacy March 28, 2016.
For personal use only. No other uses without permission. Copyright ©2016. Elsevier Inc. All rights reserved.
38 
Children with neuromuscular disorders often present
clinically as a delay in motor milestones. In many cases, it
may not be clear from the symptoms and signs whether the
etiology is central or peripheral. One of the best examples
of this predicament is that of the floppy infant, in whom
the differential diagnosis includes the entire length of the
neuraxis, from brain to muscle. In this regard, EDX studies
often are helpful in differentiating peripheral from central
etiologies and, accordingly, guiding the subsequent evalua-
tion in a useful and logical direction.
MATURATION ISSUES
When studying children, it is essential to appreciate what
is normal for what age. This is especially important when
interpreting conduction velocities and differentiating a
normal conduction velocity from axonal loss or demyelina-
tion. Most adult electromyographers who study adults are
well versed in the EDX criteria for demyelination:
• Conduction velocities less than 75% the lower limit of
normal
• Distal latencies and late responses greater than 130%
the upper limit of normal
• Conduction block, which signifies not only demyelina-
tion but acquired demyelination
However, infants and young children often have slowed
conduction velocities that would be considered in the
“demyelinating range” for adults. In most cases, this is not
because infants and young children have demyelinated
nerves; rather, they have nerves that have yet to be myeli-
nated in the first place. The process of myelination is
age dependent, beginning in utero, with nerve conduction
velocities in full-term infants approximately half that of
adult normal values. Accordingly, nerve conduction veloci­
ties of 25 to 30 m/s are normal at birth. Conduction veloc-
ity rapidly increases after birth, reaching approximately
75% of adult normal values by age 1 year, and the adult
range by age 3 to 5 years, when myelination is complete.
Accordingly, when a child is studied in the EMG laboratory,
it is essential that age-based normal control values are used
(Tables 38–1 and 38–2).
One interesting aspect of myelin maturation is often
observed during the nerve conduction studies. Many are
familiar with the fact that different white matter tracts in
594 SECTION VII Electromyography in Special Clinical Settings

Table 38–1.  Pediatric Motor Conduction Studies by Age

Median Nerve Peroneal Nerve
Age DML (ms) CV (m/s) F (ms) AMP (mV) DML (ms) CV (m/s) F (ms) AMP (mV)
7 days–l month 2.23 (0.29)* 25.43 (3.84) 16.12 (1.5) 3.00 (0.31) 2.43 (0.48) 22.43 (1.22) 22.07 (1.46) 3.06 (1.26)
1–6 months 2.21 (0.34) 34.35 (6.61) 16.89 (1.65) 7.37 (3.24) 2.25 (0.48) 35.18 (3.96) 23.11 (1.89) 5.23 (2.37)
6–12 months 2.13 (0.19) 43.57 (4.78) 17.31 (1.77) 7.67 (4.45) 2.31 (0.62) 43.55 (3.77) 25.86 (1.35) 5.41 (2.01)
1–2 years 2.04 (0.18) 48.23 (4.58) 17.44 (1.29) 8.90 (3.61) 2.29 (0.43) 51.42 (3.02) 25.98 (1.95) 5.80 (2.48)
2–4 years 2.18 (0.43) 53.59 (5.29) 17.91 (1.11) 9.55 (4.34) 2.62 (0.75) 55.73 (4.45) 29.52 (2.15) 6.10 (2.99)
4–6 years 2.27 (0.45) 56.26 (4.61) 19.44 (1.51) 10.37 (3.66) 3.01 (0.43) 56.14 (4.96) 29.98 (2.68) 7.10 (4.76)
6–14 years 2.73 (0.44) 57.32 (3.35) 23.23 (2.57) 12.37 (4.79) 3.25 (0.51) 57.05 (4.54) 34.27 (4.29) 8.15 (4.19)
*Mean (SD). DML = distal motor latency; CV = conduction velocity; F = F-latency; AMP = amplitude.
From Parano, E., Uncini, A., DeVivo, D.C., Lovelace, R.E., 1993. Electrophysiologic correlates of peripheral nervous system maturation in infancy and
childhood. J Child Neurol 8, 336–338.

Table 38–2.  Pediatric Sensory Conduction Studies by Age

Median Nerve Sural Nerve
Age CV (m/s) AMP (µV) CV (m/s) AMP (µV)
7 days–l month 22.31 (2.16)* 6.22 (1.30) 20.26 (1.55) 9.12 (3.02)
1–6 months 35.52 (6.59) 15.86 (5.18) 34.63 (5.43) 11.66 (3.57)
6–12 months 40.31 (5.23) 16.00 (5.18) 38.18 (5.00) 15.10 (8.22)
1–2 years 46.93 (5.03) 24.00 (7.36) 49.73 (5.53) 15.41 (9.98)
2–4 years 49.51 (3.34) 24.28 (5.49) 52.63 (2.96) 23.27 (6.84)
4–6 years 51.71 (5.16) 25.12 (5.22) 53.83 (4.34) 22.66 (5.42)
6–14 years 53.84 (3.26) 26.72 (9.43) 53.85 (4.19) 26.75 (6.59)
*Mean (SD); CV = conduction velocity; AMP = amplitude.
From Parano, E., Uncini, A., DeVivo, D.C., Lovelace, R.E., 1993. Electrophysiologic correlates of peripheral nervous system maturation in infancy and
childhood. J Child Neurol 8, 336–338.

the central nervous system myelinate at different times. 10 µV

Indeed, one can often use the pattern of myelination on a 2 ms
brain magnetic resonance imaging (MRI) scan to correctly
predict the age of a young child. Similarly, different fibers
in the peripheral nervous system myelinate at different
times as well. In the EMG laboratory, this often manifests
as a bifid morphology (i.e., two separate peaks) on sensory
nerve action potentials (SNAPs) in infants and children
(Figure 38–1). This bifid morphology is due to some fibers
having already been fully myelinated (the first peak),
whereas others have not and trail behind (i.e., the second
peak). It is not unusual to see bifid SNAPs between the FIGURE 38–1  Sural sensory nerve action potential in a young
ages of 3 months and 4 to 6 years. These bifid SNAPs are child. Note the bifid morphology. These bifid sensory responses are
a completely normal finding. Eventually, as the fibers in the a completely normal finding between the ages of 3 months through
4 to 6 years. They occur as different populations of fibers myelinate
second peak fully myelinate, the second peak moves to the at different times. Eventually, the group of fibers in the second peak
left and merges with the first peak. This forms a larger will fully myelinate. The second peak will move to the left and
sensory response, as is typically seen in adults. merge with the first peak to form a larger sensory response.

Downloaded from ClinicalKey.com at Univ Targu Mures Med Pharmacy March 28, 2016.
For personal use only. No other uses without permission. Copyright ©2016. Elsevier Inc. All rights reserved.
 Chapter 38 • Approach to Pediatric Electromyography 595

As with adults, the F response can be easily studied in TECHNICAL ISSUES

children. Although the F response often is thought of as
evaluating the proximal nerve segments, it assesses the
entire length of the nerve, from the stimulation point to
the spinal cord and back, and then past the stimulation
point to the muscle. Thus, the F response latency depends
not only on the conduction velocity and distal latency but
also on the length of the limb. Because infants and children
have slower conduction velocities than adults, one would
expect the F responses to be very long. However, counter-
balancing this is the very short limb length of a child com-
pared to an adult. Thus, there are two opposing influences
on the F response in children: limb length and conduction
velocity. In infants and young children, the influence of the
limb length is more overriding, resulting in F-wave latencies
that are much shorter in children than adults (typically in
the range of 16–19 ms in the upper extremities). Thus,
whenever an F response is performed on a child, it is essen-
tial to compare it to normal control values for the child’s
age or height.
The most important maturation issue for the needle
EMG portion of the examination is the size of the motor
unit. It is no surprise that the physical size of a motor unit
of a newborn is much smaller than that of an adult. Trans-
verse motor unit territory increases greatly with age,
doubling from birth to adulthood, mostly because of the
increase in individual muscle fiber size. Thus, normal motor
unit action potentials (MUAPs) in infants typically are very
small, representing the physical size of the motor unit.
Indeed, in infants, it often is difficult to differentiate normal
MUAPs from myopathic ones. This once again underscores
that when one interprets EDX findings in children, includ-
ing MUAPs, it is essential to use age-based normal control
values (Table 38–3).
A large number of unique technical issues must be kept in
mind when studying infants and children so that reliable
and accurate data can be obtained. The first important issue
is measurement of distances and its relationship to techni-
cal errors. Because a child’s limb is much smaller than an
adult’s, much shorter distances are used. When short dis-
tances are used, a small error in measurement creates a
much larger error in computed conduction velocities than
when longer distances are used. For instance, in an adult,
if the distance between the wrist and elbow is measured at
20 cm but is off by 1 cm (i.e., the true measurement is
21 cm), this results in an error of 5% when calculating a
conduction velocity. However, in a newborn baby, if the
measured distance is 7 cm but is off by 1 cm (i.e., the true
measurement is 8 cm), the error in conduction velocity
increases to 14%. Thus, one needs to be especially careful
when measuring distances in children.
Second, smaller electrodes often are needed in infants
and young children because their limbs and muscles are so
small. The typical bar electrode that has the active and
reference contacts separated by 2.5 cm often is too large
for most infants and small children (Figure 38–2). Standard
10 mm disc electrodes often will suffice for most ages,
except for newborns in which smaller electrodes generally
are needed. Likewise, the standard adult stimulator often
is too large for infants and young children because of the
size of the prongs and the distance between the cathode
and anode. Often it is preferable to use a pediatric-sized
stimulator so that the nerve of interest is more accurately
stimulated (Figure 38–3).
Because a child’s limbs are so much smaller than an
adult’s, one needs to take great care when stimulating the

Table 38–3.  Mean Motor Unit Action Potential Duration Based on Age and Muscle Group
Age of Arm Muscles (ms) Leg Muscles (ms)
Subjects
(yrs) Deltoid Biceps Triceps Thenar ADM Quad, BF Gastroc Tib Ant Per Long EDB Facial
0–4 7.9–10.1 6.4–8.2 7.2–9.3 7.1–9.1 8.3–10.6 7.2–9.2 6.4–8.2 8.0–10.2 6.8–7.4 6.3–8.1 3.7–4.7
5–9 8.0–10.8 6.5–8.8 7.3–9.9 7.2–9.8 8.4–11.4 7.3–9.9 6.5–8.8 8.1–11.0 5.9–7.9 6.4–8.7 3.8–5.1
10–14 8.1–11.2 6.6–9.1 7.5–10.3 7.3–10.1 8.5–11.7 7.4–10.2 6.6–9.1 8.2–11.3 5.9–8.2 6.5–9.0 3.9–5.3
15–19 8.6–12.2 7.0–9.9 7.9–11.2 7.8–11.0 9.0–12.8 7.8–11.1 7.0–9.9 8.7–12.3 6.3–8.9 6.9–9.8 4.1–5.7
20–29 9.5–13.2 7.7–10.7 8.7–12.1 8.5–11.9 9.9–13.8 8.6–12.0 7.7–10.7 9.6–13.3 6.9–9.6 7.6–10.6 4.4–6.2
30–39 11.1–14.9 9.0–12.1 10.2–13.7 10.0–13.4 11.6–15.6 10.1–13.5 9.0–12.1 11.2–15.1 8.1–10.9 8.9–12.0 5.2–7.1
40–49 11.8–15.7 9.6–12.8 10.9–14.5 10.7–14.2 12.4–16.5 10.7–14.3 9.6–12.8 11.9–15.9 8.6–11.5 9.5–12.7 5.6–7.4
50–59 12.8–16.7 10.4–13.6 11.8–15.4 11.5–15.1 13.4–17.5 11.6–15.2 10.4–13.6 12.9–16.9 9.4–12.2 10.3–13.5 6.0–7.9
60–69 13.3–17.3 10.8–14.1 12.2–15.9 12.0–15.7 13.9–18.2 12.1–15.8 10.8–14.1 13.4–17.5 9.7–12.7 10.7–14.0 6.3–8.2
70–79 13.7–17.7 11.1–14.4 12.5–16.3 12.3–16.0 14.3–18.6 12.4–16.1 11.1–14.4 13.8–17.9 10.0–13.0 11.0–14.3 6.5–8.3
ADM, abductor digiti minimi; BF, biceps femoris; EDB, extensor digitorum brevis; Gastroc, gastrocnemius; Per long, peroneus longus; Quad, quadriceps;
Tib ant, tibialis anterior.
Reprinted with permission from Buchthal, F., Rosenfalck, P., 1955. Action potential parameters in different human muscles. Acta Psych Neurol Scand.
Munsgaard International Publishers Ltd, Copenhagen, Denmark.

Downloaded from ClinicalKey.com at Univ Targu Mures Med Pharmacy March 28, 2016.
For personal use only. No other uses without permission. Copyright ©2016. Elsevier Inc. All rights reserved.
596 SECTION VII Electromyography in Special Clinical Settings
Bar 10 mm
electrode disc electrodes
FIGURE 38–2  Pediatric electrodiagnostic studies and recording
electrode size. The standard bar electrode (left) and 10 mm disc
electrodes (middle) are compared to the size of an infant’s hand
(right). Smaller electrodes may be needed in infants and young
children because their limbs and muscles are so small. Standard
10 mm disc electrodes often will suffice for most age groups,
including infants. In newborns, however, smaller electrodes are
needed. Other standard electrodes, like the bar electrode, are too
large for a newborn or infant’s hand.
Pediatric Standard Infant 5 year-old Adult
stimulator stimulator
FIGURE 38–3  Pediatric electrodiagnostic studies and stimulator
size. The standard stimulator can be used for adults and most
children. However, for infants and young children, it is preferable to
use a pediatric-sized stimulator so that the nerve of interest is more
accurately stimulated.
nerves. The stimulus intensity needs to be kept as low as
possible, for patient cooperation and tolerance but also to
prevent co-stimulation of nearby nerves. Co-stimulation of
nerves is much more likely to occur in a young infant or
child than in an adult, even at low intensities, because of
the small size of the limb and the close physical proximity
of the nerves to each other.
During the needle EMG examination, additional techni-
cal issues arise. Because the physical size of the motor units
Downloaded from ClinicalKey.com at Univ Targu Mures Med Pharmacy March 28, 2016.
For personal use only. No other uses without permission. Copyright ©2016. Elsevier Inc. All rights reserved.
in children is quite small, it is often very difficult, even for
the most experienced pediatric electromyographer, to dif-
ferentiate normal MUAPs from myopathic MUAPs, espe-
cially in infants. Decreased recruitment and large MUAPs,
as seen in neuropathic conditions, are much more straight-
forward and easier to appreciate in infants and children
than normal or myopathic MUAPs in this population.
Because individual muscle fibers are so small in infants
and children, another common problem that arises in pedi­
atric electromyography is the differentiation between fibril­
lation potentials and endplate spikes. The endplate zone in
infants takes up a disproportionately large territory of the
muscle compared with adults. Thus, it is not uncommon
to encounter endplate potentials when studying pediatric
patients. Endplate spikes can easily mimic fibrillation
potentials. One needs to pay especially close attention to
the firing pattern (regular vs. irregular) and the initial wave-
form deflection (positive vs. negative) to properly differen-
tiate fibrillation potentials from endplate spikes. Because
fibrillation potentials signify active denervation, it is essen-
tial not to mistake endplate spikes for fibrillation potentials,
especially in the pediatric population, where such findings
may portend a particularly grave diagnosis, such as infantile
spinal muscular atrophy (Werdnig–Hoffmann disease).
APPROACH TO THE CHILD
AS A PATIENT
Although many adults are apprehensive of EDX studies,
most tolerate the study well, with minimal discomfort. In
adults, explaining the test in advance and as it proceeds
is often one of the most helpful ways of allaying any fears
and creating good patient rapport. However, a different
approach must be taken to allay fears and create rapport
with an infant or child in the EMG laboratory. As most
children are accompanied by their parent(s), it is often
extremely helpful to have a parent in the room with the
child. The parent can help comfort the child and be a valu-
able asset to the electromyographer. The electromyogra-
pher also might consider removing his or her white coat
before entering the examination room. Speaking with the
child in a supportive and comforting manner, using uncom-
plicated words and phrases, will help allay the child’s fears.
Of course, the task is much more difficult in infants, who
cannot understand the situation, and in these cases having
a parent in the room is extremely valuable.
There are a few helpful techniques that can be used
with children to gain their cooperation. When performing
the nerve conduction studies, the electromyographer can
explain to the child that the stimulator will feel like a tap,
buzz, or static electricity, similar to when he or she rubs
the feet along the floor and then touches the refrigerator.
It is best to avoid the word “shock” when explaining the
nerve conduction part of the study, because the term likely
has negative connotations for both children and adults. One
extremely effective maneuver is to have the child hold the
stimulator and stimulate the examiner’s median nerve at

the wrist, using a low-stimulus current. In this way, the
child can see the muscle twitch. More importantly, the
child will see that the examiner is not distressed by
the experience (hopefully). In children aged 5 through 10
years, we routinely have them stimulate our own nerves
before we begin the study. One will find that the parent in
the room often is interested in knowing what the stimulator
feels like on themselves. Regarding the needle part of the
test, the word “needle” should always be avoided. No one
likes needles, including children. Children are very familiar
with needles, usually receiving one or more vaccinations
almost every time they visit their pediatrician. It is best to
use the word “electrode” or “microphone” when describing
the needle part of the examination. If a child is told that a
very small microphone is going to be put into his or her
muscle so that he or she will be able to hear the muscles
along with you, the child may become very interested and
engaged in the test.
The most difficult age for EDX studies is between the ages
of 2 and 6 years. In the infant, who cannot understand and
who also cannot move around very much, EDX studies
usually can be done fairly easily and quickly, with minimal
discomfort to the infant, with an assistant helping immo-
bilize the limb being studied. However, in rambunctious
toddlers, EDX studies can be very difficult without their
cooperation. Indeed, in this age group, conscious sedation
often is very helpful.
In the past, a mild sedative such as chloral hydrate was
often used. This form of sedation usually was inadequate,
with the child often sleeping well on the ride home after
the study but not during the study. In the modern day,
conscious sedation with propofol (Diprivan), under the
supervision of an anesthesiologist, can be used to obtain
good data with minimal discomfort to the child. Propofol
is an intravenous sedative–hypnotic agent used for induc-
tion of anesthesia or for sedation. Its major advantage is
that it produces hypnosis rapidly, usually within 40 seconds
from the start of the injection. As with other rapidly acting
intravenous anesthetic agents, the half-time of the blood–
brain equilibration is approximately 1 to 3 minutes. While
the child is sedated with propofol, nerve conduction studies
and/or repetitive nerve stimulation studies can be per-
formed easily. Likewise, the needle EMG study can be
performed, looking for abnormal spontaneous activity,
while the child is sedated. The propofol then can be turned
down, and, as the child is coming out of the sedation,
MUAPs can be analyzed (Figure 38–4).
Pediatric electromyography nevertheless remains a chal-
lenge, even if these recommendations are followed. The
more experience one has with children, the easier the
testing goes. In pediatric electromyography, more than in
any other situation, it is important to always follow the
Willie Sutton rule: Go where the money is! One needs to
carefully choose the nerves and muscles to study based on
the following:
• Which studies are essential to help support or exclude
a diagnosis
Downloaded from ClinicalKey.com at Univ Targu Mures Med Pharmacy March 28, 2016.
For personal use only. No other uses without permission. Copyright ©2016. Elsevier Inc. All rights reserved.
Chapter 38 • Approach to Pediatric Electromyography 597
1.00 Target plasma concentration
plasma propofol concentration
0.75
(ug/mL)
Awakening
0.50
Recovery after 1 hour infusion
0.25
0.00
0 20 40 60 80
Minutes
FIGURE 38–4  Propofol plasma concentration kinetics. Under the
direction of an anesthesiologist, propofol can be used successfully to
sedate young children undergoing electrodiagnostic studies. Its major
advantage is that it produces a rapid hypnosis. Upon stopping the
infusion, the concentration rapidly declines and the child begins to
awaken within a few minutes. While the child is sedated, nerve
conduction studies, repetitive nerve stimulation studies, and needle
electromyography (assessing spontaneous activity) can be performed.
As the child begins to awaken, motor unit action potentials can be
analyzed.
• Which nerve conduction studies are the fastest and
easiest to perform
• Which muscles are the easiest to activate and the
least painful to study
For example, the median motor nerve is much easier to
stimulate and record than the tibial motor nerve, which is
difficult and painful to stimulate behind the popliteal fossa.
Likewise, it is important to choose muscles that are less
painful and easier to activate than others. For instance, the
first dorsal interosseous (FDI) and the abductor pollicis
brevis (APB) both are distal upper extremity C8–T1-inner-
vated muscles. However, the FDI is much less painful to
sample than the APB. In children, it is always best to pur-
posefully choose the least painful muscles to examine,
unless it is absolutely necessary to examine a muscle that
is known to be painful. In addition, it is important to choose
muscles that are easy to activate. In children who cannot
cooperate, it often is useful to choose muscles that can be
activated by withdrawing to a sensory stimulus. For instance,
tickling the foot will result in contraction of the tibialis
anterior and hamstring muscles as the child reflexively pulls
his or her leg away.
One of the most important rules in pediatric electromyo­
graphy is: “Take what you can get, when you can get it!”
When examining an adult, the electromyographer is accus-
tomed to placing the needle electrode in the muscle,
looking first at insertional and spontaneous activity, and
then changing the gain to 200 µV per division while having
the patient contract to look at the MUAPs. In a child, if
one puts the needle electrode into a muscle and MUAPs
are firing, do not try to get the child to relax the muscle.
It is much more productive to quickly change the sensitiv-
ity to 200 µV per division and look at the MUAPs while
they are firing, because you might not get another chance!
598 SECTION VII Electromyography in Special Clinical Settings

Do not expect to follow the same regular routine in a child
that you normally would follow in an adult.
GOALS OF THE PEDIATRIC
ELECTRODIAGNOSTIC
EXAMINATION
The goals of the EDX study in infants and children are
similar to those in adults. The first goal is to discern if a
neuromuscular disorder is present. Differentiating between
a central and peripheral cause of weakness is of prime
importance to the referring physician. If the problem is
peripheral, the next goal is to determine if the pathology
is neuropathic, myopathic, or due to a disorder of the
neuromuscular junction. This differentiation then allows
for a more efficient and logical use of further laboratory
testing. If the condition is neuropathic, the next goal is to
determine if motor, sensory, or a combination of fibers is
involved. This relies primarily on whether SNAPs are
present, reduced, or absent. Take the example of a young
child with diffuse denervation and reinnervation on needle
EMG associated with low motor amplitudes on nerve con-
duction studies. Taken together, these findings denote a
neuropathic process. If the SNAPs are normal, then the
disorder most likely localizes to the anterior horn cells.
Although these findings also might be seen in a pure motor
neuropathy, this would be very unlikely in an infant or
child. On the other hand, if the SNAPs are abnormal, then
a peripheral neuropathy likely is present, which has a very
different differential diagnosis and prognosis. If a periph-
eral neuropathy is present, the next important piece of
information to discern from the EDX study is whether or
not the pathology is demyelinating. Because so many pedi-
atric peripheral neuropathies are genetic in nature and
because the demyelinating forms of Charcot–Marie–Tooth

Table 38–4.  Recommended Approach to Childhood Neuromuscular Disorders

Diagnostic Test/Procedure
Suspected Clinical Diagnosis Option: 1st 2nd 3rd
Duchenne–Becker MD DNA MBx
1
LGMDs DNA MBx EMG/NCS2
Congenital muscular dystrophies MBx DNA EMG/NCS2
Emery–Dreifuss MD DNA MBx EMG/NCS2
FSH MD DNA MBx EMG/NCS3
MyD DNA EMG/NCS
Periodic paralysis/myotonias DNA EMG/NCS
Metabolic MBx DNA EMG/NCS3
Congenital myopathies MBx DNA4 EMG/NCS5
DM/PM MRI MBx EMG/NCS3
Indeterminate proximal weakness EMG/NCS RMNS MBx/DNA
SMA DNA EMG/NCS MBx6
CIDP EMG/NCS CSF NBx
AIDP (GBS) CSF EMG/NCS
HMSNs EMG/NCS DNA
Neuromuscular transmission disorders EMG/NCS RMNS Antibodies/DNA7
AIDP, acute inflammatory demyelinating polyneuropathy; BMD, Becker muscular dystrophy; CIDP, chronic inflammatory demyelinating polyneuropathy;
CSF, cerebrospinal fluid examination; DM/PM, dermatomyositis/polymyositis; DMD, Duchenne muscular dystrophy; DNA, deoxyribonucleic acid/genetic
testing; EMG, electromyography; FSH, facioscapulohumeral; GBS, Guillain–Barré syndrome; HMSNs, hereditary motor and sensory neuropathies; LGMD,
limb-girdle muscular dystrophy; MBx, muscle biopsy; MD, muscular dystrophy; MRI, magnetic resonance imaging; MyD, myotonic dystrophy; NBx, nerve
biopsy; NCS, nerve conduction studies; RMNS, repetitive motor nerve stimulation; SMA, spinal muscular atrophy.
Note that even in the era of molecular diagnostics, electromyography continues to play a prominent role in the evaluation of pediatric neuromuscular
disorders.
1. DNA testing is now available for many of the LGMDs. In addition, DNA testing is helpful in a limb-girdle phenotype to also exclude DMD/BMD;
2. In atypical, sporadic cases with low creatine kinase values;
3. Optional;
4. If available;
5. In certain cases, EMG/NCS may be the first option;
6. If EMG/NCS consistent with SMA but DNA test is negative;
7. For congenital myasthenic syndromes.
From Darras, B.T., Jones, H.R. Jr., 2000. Diagnosis of pediatric neuromuscular disorders in the era of DNA analysis. Pediatr Neurol 23, 289–300, with permission.

Downloaded from ClinicalKey.com at Univ Targu Mures Med Pharmacy March 28, 2016.
For personal use only. No other uses without permission. Copyright ©2016. Elsevier Inc. All rights reserved.

disease are the most common, the presence of conduction
velocities in the demyelinating range has great importance.
Of course, there also are instances of acquired demye­
linating neuropathies in children, which can usually be
distinguished from genetic forms of demyelinating neu-
ropathy using the same guidelines that apply to adults (see
Chapter 26).
In general, there is a very good correlation between the
results of EDX studies and the final diagnosis. This is espe-
cially true for neuropathic disorders (i.e., anterior horn cell
disorders and peripheral neuropathy). They are also helpful
but not as good for myopathic disorders, especially in
children younger than age 2. As noted earlier, motor units
in young children are normally quite small, making the
differentiation between normal and myopathic motor
unit action potentials very demanding. In addition, some
myopathies are fairly “bland” on needle EMG, most often
the congenital myopathies. This is in contradistinction to
the muscular dystrophies and myositis which are much
more easily recognized as myopathic on needle EMG. One
might think that in the present era of molecular genetics
wherein DNA and other forms of genetic analysis are avail-
able for many of the inherited neuromuscular conditions
(e.g., spinal muscular atrophy, many of the muscular dys-
trophies, and many forms of Charcot–Marie–Tooth disease),
EDX studies would play less of a role than in the past. This
is true for the infant or child who has a classic phenotype
of a well-known inherited disorder. In these cases, espe-
cially if there is a positive family history, the diagnosis can
often be confirmed by genetic testing, without the need
for EDX studies. However, this remains a minority of
the cases. In the evaluation of a child with weakness or a
delay in motor milestones, EDX studies still play a major
role in guiding the evaluation process in a logical and
efficient manner, with occasional diagnoses made directly
from data obtained from EDX studies (Table 38–4).
For instance, Dejerine–Sottas syndrome (DSS) is a term
applied to a group of genetically heterogeneous demyelinat-
ing neuropathies that typically present in infancy or early
childhood. DSS can easily mimic the clinical presentation
of Werdnig–Hoffman (spinal muscular atrophy type 1).
Downloaded from ClinicalKey.com at Univ Targu Mures Med Pharmacy March 28, 2016.
For personal use only. No other uses without permission. Copyright ©2016. Elsevier Inc. All rights reserved.
Chapter 38 • Approach to Pediatric Electromyography 599
However, DSS is associated with the slowest conduction
velocities ever recorded in humans, typically less than
12 m/s and usually less than 6 m/s. The finding of such a
slowed conduction velocity on nerve conduction studies
will immediately point to the diagnosis of DSS. Afterward,
appropriate genetic testing can be undertaken looking for
the known mutations associated with DSS, which include
mutations of the P0, MP22, and EGR2 genes, among
others.
Without doubt, the pediatric EDX study is much more
challenging and difficult to perform than a similar study in
an adult. However, being aware of the unique maturational
and technical issues associated with studying infants and
children and approaching the examination with a different
philosophy will offer the electromyographer the same kinds
of useful information that can be obtained in adults.
Suggested Readings
Darras, B.T., Jones, H.R., 2000. Diagnosis of pediatric
neuromuscular disorders in the era of DNA analysis.
Pediatr Neurol 23, 289–300.
Gabreels-Festen, A., 2002. Dejerine–Sottas syndrome grown
to maturity: overview of genetic and morphological
heterogeneity and follow-up of 25 patients. J Anat 200,
341–356.
Hellmann, M., von Kleist-Retzow, J.C., Haupt, W.F., et al.,
2005. Diagnostic value of electromyography in children
and adolescents. J Clin Neurophysiol 22 (1), 43–48.
Jones, H.R., Bolton, C.F., Harper, C.M., et al., 1996.
Pediatric clinical electromyography. Lippincott Williams &
Wilkins, Philadelphia.
Jones, H.R. Jr, De Vivo, D.C., Darras, B.T. (Eds.), 2003.
Neuromuscular disorders of infancy, childhood, and
adolescence: a clinician’s approach. Butterworth
Heinemann, Philadelphia.
Parano, E., Uncini, A., DeVivo, D.C., et al., 1993.
Electrophysiologic correlates of peripheral nervous system
maturation in infancy and childhood. J Child Neurol 8,
336–338.
Rabie, M., Jossiphov, J., Nevo, Y., 2007. Electromyography
(EMG) accuracy compared to muscle biopsy in childhood.
Child Neurol 22 (7), 803–808.