You are on page 1of 14

The Neurologic Exam in Neonates

and Toddlers 2
Alison S. May and Sotirios T. Keros

Introduction The History

Performing a comprehensive neurological exam- As always, obtaining a solid history of present


ination in children is sometimes considered a illness is essential for directing the physical exam
challenge by non-neurologists. A neurologic and establishing a diagnosis, and one is often
exam which tests all aspects of all neurologic able to narrow the differential diagnosis substan-
modalities can quite literally take several hours tially based on history alone. As most people who
to perform. On the other hand, a very good, thor- work with children have already learned, simple
ough neurologic exam which yields substantial observation of the child while obtaining the his-
relevant information can be performed in under tory can also influence one’s approach toward
5 min. Adherence to a systematic framework or obtaining information.
approach to the examination, appropriate for the
age and abilities of the child, can be extremely
helpful in simplifying the basic questions: Is this Birth History
child normal, and if not, why not, and how do I
describe it? This chapter will focus on how to It is likely not necessary to remind anyone with a
perform some of the routine elements of the neu- background in pediatrics that any history in chil-
rologic examination with tips on how to tailor the dren should include details of the pregnancy and
exam for various age groups. birth. Many neurologic disorders, whether
genetic or acquired, can begin in pregnancy.
Complications in pregnancy, such as growth
restriction, failure to progress, fetal distress, or
A.S. May, M.D. prolonged labor, may indicate antenatal disorders
Department of Pediatrics, New York Presbyterian rather than any process which began as a result of
Hospital, 525 E. 68TH ST BOX 91, New York, the actual birth process itself. One should evalu-
NY 10021, USA
ate for maternal antenatal factors such as illnesses
S.T. Keros, M.D., Ph.D. (*) and teratogenic exposures. Of course, the gesta-
Pediatric Neurology, Weill Cornell Medical College,
tional age at which the child was born will also
525 E. 68th St, Box 91, New York, NY 10021, USA
help establish a developmentally appropriate
Sanford Children’s Hospital, University of South
norm. If known, Apgar scores may also be infor-
Dakota Sanford School of Medicine, Sioux Falls,
SD 57105, USA mative and give a general clue as to the onset or
e-mail: Sok2005@med.cornell.edu timing of childhood disorders.

© Springer Science+Business Media New York 2017 11


J.P. Greenfield, C.B. Long (eds.), Common Neurosurgical Conditions in the Pediatric Practice,
DOI 10.1007/978-1-4939-3807-0_2
12 A.S. May and S.T. Keros

Developmental History and the cerebrospinal fluid. To measure the head


circumference, a tape measure is placed on the
Typically, developmental milestones are divided occiput posteriorly and placed above the eye-
into three broad domains—motor, language, and brows anteriorly or along the largest protrusion of
social. Assessing the current developmental the forehead. Ideally this measurement is taken a
milestones achieved is essential in evaluating few times and averaged, as it is can be difficult to
mental status and cognition. Knowing when accurately measure the head circumference, and
some key milestones, such as first words or first there is often variation between measurements.
steps, were reached will help to classify the Circumference measurements should be plotted
severity of any delays. Determining the pattern on an appropriate growth chart. Macrocephaly is
and timing of any developmental problem is nec- typically defined as an occipitofrontal head cir-
essary to classify it as static (such as might be cumference greater than the 97th percentile for
seen in cerebral palsy), progressive (as might be age, while microcephaly refers to measurements
seen in some mitochondrial disorders), or regres- less than the 2nd or 3rd percentile.
sive (as seen in Rett syndrome, some leukodys- It is critical to remember that any rapid
trophies and Landau-Kleffner syndrome, to list a changes or progressive trends in percentile are
few examples). Additional considerations in likely more important than any absolute value at
assessing developmental norms are discussed in a single point in time.
the “Mental Status” section. Rapid increases in head circumference may be
the first indication of hydrocephalus. On the other
hand, rates of head growth which do not keep
The General Exam pace with weight or length may represent a brain
which is not growing as expected. In cases of sys-
Although clichéd, it bears repeating that the temic disease such as malnourishment, head size
majority of the neurological examination can be is relatively preserved compared to length and
performed simply by paying careful attention to a weight. Please refer to Chap. 14 for a full discus-
child’s affect, behaviors, and natural or spontane- sion of the evaluation of a large head.
ous movements. Of course, this does not obviate Next, the anterior and posterior fontanelles
the need for a systematic, methodical approach to should be assessed. The anterior fontanelles
a complete neurologic exam when the situation should be evaluated with the child in an upright
warrants. There are many neurologic disorders position. An anterior fontanelle which is bulging
that have systemic “non-neurologic” involve- or firm can be a sign of increased intracranial
ment which are evident during the general physi- pressure. The anterior fontanelle typically closes
cal exam. This chapter will first point out a few between 7 to 19 months of age, and several con-
key considerations to note while performing a ditions are associated with early or delayed clo-
general exam, which are relevant to neurologic sure. However, early or late fontanelle closure
disorders, and then proceed to address the con- without other abnormalities in the exam is rarely
ventional neurologic examination. a cause of concern. The posterior fontanelle can
be closed as early as birth and otherwise closes
by 2 months of age.
The Head The cranial sutures, the edges of the bone
plates that form the skull, should also be assessed
The first assessment of the head is to determine its while examining the head. Craniosynostosis, the
size. This measurement is a way to evaluate the premature closure of these sutures, will affect the
status of the central nervous system in the new- size and shape of the head. Plagiocephaly (liter-
born and early childhood period, because head ally means “oblique head”) refers to a flattening
size is a proxy for the overall volume of the brain of a portion of the skull. The most commonly
2 The Neurologic Exam in Neonates and Toddlers 13

seen form is positional plagiocephaly, where the rigidity is often not present in cases of meningi-
occipital region is flattened (usually toward the tis, particularly in children.
lateral side) and the ipsilateral frontal area is
prominent due to forward protrusion. The inci-
dence of positional plagiocephaly has increased Cardiovascular and Abdomen
as a result of the “back-to-sleep” campaign.
Positional plagiocephaly is primarily a cosmetic Auscultation of the heart or other large vessels
issue, which does not affect brain development. such as the carotid arteries, descending aorta, or
Craniosynostosis and plagiocephaly are dis- renal arteries can reveal murmurs or bruits which
cussed in more detail in Chap. 6. may be a risk factor for stoke from causes such as
embolism, renovascular hypertension arising
from fibromuscular dysplasia, or Takayasu arteri-
The Face tis. Organomegaly, specifically hepatospleno-
megaly, can be present in many storage diseases.
The presence of dysmorphic facial features, if
any, may be suggestive of genetic syndromes.
While 15 % of normal newborns may have one The Skin
dysmorphic feature, the presence of two or more
of such features is much less common and is The skin and the central nervous system both
associated with increased risk of a clinically sig- develop from the ectoderm during embryogene-
nificant anomaly. Some constellations of facial sis, and many neurologic diseases are associated
features are readily identified by most people with dermatologic findings. There are several
(e.g., the classic facies in Down syndrome), but classic neurocutaneous disorders where the diag-
many abnormal facial features can be subtle and nosis can often be made simply from skin find-
not easily recognized. It is helpful to systemati- ings. Café au lait spots, axillary or inguinal
cally examine and even measure several facial freckling, and neurofibromas are markers of neu-
features, as there are well-established norms for rofibromatosis Type 1. Ash leaf spots or hypopig-
comparison. Typically, this type of detailed mented patches, adenoma sebaceum, and
facial analysis is performed by geneticists. shagreen patches are lesions seen in children
Interestingly, computer automated-dysmor- with tuberous sclerosis. Cutaneous vascular
phometry is a growing field of research, and lesions, such as capillary malformations involv-
there are case reports where this technique has ing the ophthalmic region of the trigeminal nerve,
revealed a genetic diagnosis. are associated with Sturge-Weber syndrome.
Additional information on neurocutaneous disor-
ders is provided in Chap. 7.
The Neck

The neck should be examined to assess for full The Spine and Back
range of motion and absence of rigidity or asym-
metry, particularly if there are abnormalities of Abnormalities noted in the back or spine, such as
the head or head shape. Infant torticollis, which discolorations, dimples, or tufts, may be clues to
results in twisting of the head due to a shortened an underlying problem with the spinal cord.
sternocleidomastoid muscle, is a common cause Alternately, purely bony problems with the spine,
of positional plagiocephaly. Nuchal rigidity is of such as hemivertebrae, can cause secondary spi-
course a concerning sign which may signify nal cord injuries through severe scoliosis or nar-
meningeal irritation due to infection or other rowing of the spine. Please see Chaps. 8, 9, and
causes and should be assessed for in any ill 19 for more thorough discussion of the back,
appearing child, keeping in mind that nuchal spine, and spinal cord disorders.
14 A.S. May and S.T. Keros

The Neurologic Exam number pad. Many 4- or 5-year-old children can


remember and instantly repeat at least four digits.
The neurological exam is often presented in a Basic language skills should also be evaluated,
“head to toe” format. With infants and children, including investigation of receptive language, for
of course, the examiner does not always have the example, understanding and following com-
luxury of focused patient cooperation as he or she mands, and expressive language, such as sponta-
marches down a preset list of areas and functions neous and provoked speech, naming, repetition,
to be examined. As always, first perform which- etc. Reading and writing skills are other compo-
ever parts of the examination the patient will nents of a language examination which can be
readily allow. One strategy is to initially assess tested in school-age children.
for tone, range of motion, and deep tendon In neonates and premature babies, the mental
reflexes, while a child is resting or otherwise status exam is essentially restricted to evaluating
calm. If at any point there is crying or screaming, for gestational-age-appropriate level of alertness
this is a good time to assess the cranial nerves by and “general movements,” which describe the
observing for facial strength and symmetry and highly stereotypical patterns of motions which
palate elevation, for example. Also, an uncoop- neonates will do while awake. Before 28 weeks’
erative child’s limb strength is usually easily gestational age, it is very difficult to note discrete
assessed by the vigorousness with which the times of wakefulness. By about 28 weeks, one
child attempts to evade or terminate the exam. can observe that a gentle movement will cause
arousals in the infant. At 32 weeks, eyes begin to
open spontaneously and may stay open for
Mental Status extended periods of time. Around 36 weeks one
begins to see progressively increased periods of
The mental status and its examination are obvi- wakefulness and alertness, with vigorous crying.
ously dependent on the child’s age. By about age By term, there should be clear attention to visual
6, one can generally assess mental status and and auditory stimuli.
cognition using a similar approach as used in In infants between 1 and 6 months of age, the
adults. For completeness, a brief review of the mental status is still primarily an evaluation of
mental status for older children will be described. alertness and attentiveness, but which now can be
The key components of mental status include the also assessed with the motor behaviors which
level of arousal, orientation, attention, memory, make up the traditional milestones commonly
language, and higher cognitive functions. The assessed in children. For example, facial tracking
level of arousal is typically graded or classified requires sustained wakefulness and sustained
(from best to worst) as awake and alert, drowsy, attention, in addition to an intact visual system
confused, lethargic, obtunded, stuporous, or and functioning cranial nerves. Thus, in the
comatose. Orientation states range from fully ori- younger children, the developmental milestones
ented (knowing the date, location, the people in do not neatly fit into any one category of a neuro-
the room) to complete confusion. Standard defi- logic exam and blur the lines between mental sta-
nitions of orientation do not apply to infants and tus, cognition, and motor component.
toddlers, of course. Memory, which includes both
short-term and long-term memory, can be
assessed with a standard test of recall of named Cranial Nerves
objects after a few minutes or asking about activi-
ties performed the day before or perhaps the pre- Although the 12 cranial nerves can be individually
vious summer. Attention is commonly assessed evaluated in an order child upon request, in
with a digit span test. Give the child a list of dig- younger children these are often evaluated with
its to repeat verbally or to dial on a telephone observation of spontaneous or provoked responses.
2 The Neurologic Exam in Neonates and Toddlers 15

I: Olfactory Nerve and place your palms with fingers extended


This nerve mediates the sense of smell. This toward the patient in the area of the visual field
nerve is rarely tested even in adults and has little you wish to test. Ask the child to look at your
utility for testing in a child unless a deficit is nose and to point or look if he or she sees the
somehow otherwise suspected. movement when you briefly flex or wiggle just
your index finger a few times. This method of
II: The Optic Nerve visual field testing is preferred to finger counting
CN II transmits visual inputs from the retina to or bringing in wiggling fingers from the periph-
the areas of the brain which control reflexive ery, as many children will reflexively look toward
movements and unconscious perception (i.e., the end of any outstretched arm and thus requires
pupillary light reflexes, blink to threat, tracking repeated attempts and urging not to look to the
and pursuit mechanisms) as well as the conscious sides and anticipate the stimulus. Testing four
perception of light. quadrants in each eye is usually sufficient,
The most direct evaluation of the optic nerve remembering that visual field deficits are not nec-
is to assess for pupillary light reflex, which essarily peripheral and can also be patchy.
requires the proper functioning of the optic In younger children who cannot participate in
nerve to transmit the light information which confrontational visual field testing, an effective
hits the retina and cranial nerve III which medi- method is to hold an interesting object, such as a
ates the pupillary constriction. The direct colorful toy, with both hands behind the examin-
response describes ipsilateral constriction of the ers head. Then, simultaneously bring the object
pupil, while the consensual response is the con- out into one quadrant of the visual fiend and an
striction which also occurs in the contralateral empty hand in the opposing quadrant. If the child
eye. Pupillary light reflexes should be apparent can see the object, then he or she will look toward
by 32–35 weeks’ gestational age but can be dif- the more interesting stimulus, in this case the toy.
ficult to assess in infants because the pupils are In the youngest children, a “blink-to-threat” tech-
relatively small relative to the size of the iris at nique can be used. To do this the examiner brings
this age. an object such as a fist rapidly toward the eye.
By term, infants should be able to fixate and This is repeated, coming from different direc-
follow with their eyes, and this tracking response tions while looking for a blink response. Note
strengthens in the first 2–3 months of life. that this test can frequently be unreliable, as air
The ideal subject to test for tracking is a movements from an approaching hand or other
human face held 8–12 in from the child. An easy object may stimulate a blink response. In addi-
way to test for tracking is to hold the child in your tion, failure to blink is not necessarily an indica-
outstretched arms, facing you, as you rotate him tion of lack of sight, so one must use caution in
or her around. It is important to note that tracking over-interpreting the results of blink-to-threat
at this age is likely due to involuntary responses testing.
and deep brain structures and does not necessar- Finally, the fundoscopic exam is a method
ily involve higher cortical areas such as primary which directly visualizes the retinal portion of the
visual cortex. optic nerve. This can be very challenging in chil-
Visual field evaluation is not classically a test dren and a discussion of fundoscopy is beyond
of optic nerve integrity, although it is reasonable the scope of this chapter. If any visual deficits are
to include a discussion of visual fields in this sec- suspected in a child, a referral to a pediatric
tion. Visual field testing may identify focal reti- ophthalmologist or neuro-ophthalmologist is
nal deficits as well as brain lesions involving any suggested for a detailed examination and dilated
part of the visual pathway. In cooperative chil- fundoscopy. Chapter 12 will give an in-depth
dren (and adults), the best method is to position description of the ophthalmologic exam in
yourself at arms’ length away from the patient children.
16 A.S. May and S.T. Keros

III/IV/VI: The Oculomotor Nerve, of the affected eye. The most common cause of
the Trochlear Nerve, and the Abducens trochlear nerve damage is head trauma. Incomplete
Nerve abduction of an eye is usually due to weakness of
The oculomotor nerve, the trochlear nerve, and the lateral rectus which is innervated by the abdu-
the abducens nerve are responsible for the move- cens nerve. One cause of abducens injury is
ment of the eyes. The oculomotor nerve contains increased intracranial pressure due to brain edema
the fibers which mediate the efferent papillary or hydrocephalus. Also, Duane syndrome is a
reflex and elevate the eyelid, in addition to being form of congenital abducens nerve malfunction
the nerve which innervates most of the muscles (occasionally also involving other cranial nerves),
which control eye movements (the superior rec- which limits eye mobility but is typically benign
tus, inferior rectus, medial rectus, and inferior and does not result in overt visual deficits.
oblique). The trochlear nerve controls the supe- Horner’s syndrome is a constellation of findings,
rior oblique muscle, which has a complicated which include miosis (pupillary constriction),
function which varies depending on the direction ptosis, and anhidrosis on the same side of the face,
of gaze, but is essentially responsible for intort- and is frequently caused by disruption of sympa-
ing and depressing the eye, while the abducens thetic innervation which ascends along the carotid
nerve controls the lateral rectus which abducts artery. However, Horner’s syndrome can also
the eye for lateral movements. arise from a central brain or spinal disorder.
To formally test for the integrity of these Parinaud syndrome results in an impairment
nerves and the associated muscles, assess for full of upward gaze, often accompanied by eyelid
range of motion in the horizontal and vertical retraction and pupillary abnormalities. Parinaud
directions. Testing the “diagonals” increases the syndrome is the result of a lesion or compression
sensitivity of noting any deficits, because the of the pretectal area in the dorsal midbrain,
extraocular muscles do not attach to the globe at which is a common location for pediatric neo-
perfect right angles. If limited range of motion or plasms. Other causes of this syndrome include
disconjugate gaze is noted in any direction, each obstructive hydrocephalus or direct injury due to
eye can then be evaluated separately in an attempt ischemia or hemorrhage.
to determine which eye is the abnormal one. In
older children, inquiring about diplopia is impor- V: The Trigeminal Nerve
tant, as patient self-reporting can be more sensi- The trigeminal nerve controls sensation of the
tive than directional testing. In infants, use a face as well as the muscles of mastication. Facial
strong stimulus such as a toy or your own face in sensation in older children can be tested by
an attempt to have them follow, while you assess applying a stimulus such as light touch to each
eye movements. In newborns, using the “doll’s division of the trigeminal nerve: the ophthalmic
eye” reflex can be used to assess horizontal eye branch of the forehead, the maxillary branchor
movements. To perform this test, also known as (the cheek), and the mandibular branch (the
the oculocephalic reflex, the head is turned some- chin). In infants, tickling or stroking the face, for
what quickly but gently to one side. The move- example, with a cotton swab on one side of the
ment should result in a temporary deviation of nose, cheek, or lip, should elicit a rooting-like
both eyes in a direction opposite to the direction motor response toward the side of the face that
of turning. The doll’s reflex is typically present as was stimulated. The trigeminal nerve can also be
early as 25 weeks of gestation. tested by eliciting the corneal reflex. A very light
There are several clinically important eye touch to the cornea, such as with a wisp of cotton,
abnormalities that are worth noting. Weakness of should trigger a bilateral blink reflex. The sensa-
the superior oblique muscle or trochlear nerve tion is mediated by the ophthalmic branch, while
will result in a compensatory head tilt in many the motor response arrives via the facial nerve.
children, in order to prevent the diplopia which Chewing movements are mediated by the
results from the abnormal elevation (hypertropia) muscles of mastication which are innervated by
2 The Neurologic Exam in Neonates and Toddlers 17

the mandibular branch of V. Opening the jaw is sensorineural hearing loss arising from damage
due to the action of the external pterygoids while to the cochlea or eighth nerve. The Rinne test is
closing the jaw is due to the masseter and tempo- performed by holding the base of the vibrating
ralis. In neonates the muscles of mastication can tuning fork against the mastoid process of the
be tested indirectly by evaluation of sucking abnormal ear. When the subject no longer per-
strength and control and more directly by allow- ceives sound, the vibrating end of the tuning fork
ing the infant to bite on your fingers. is then placed just outside the ear canal. If the
tuning fork can now be heard, this signifies a
VII: The Facial Nerve “positive test” and suggests sensorineural hear-
The facial nerve controls the muscles of facial ing loss. If the tuning fork cannot be heard after
expression and also mediates taste on the anterior removing it from the mastoid, the test is negative
two thirds of the tongue. In cooperative children, and suggests a conductive hearing loss. Note that
facial nerve integrity can be demonstrated by not- in normal ears without hearing loss, the normal
ing strong eye closure, wrinkling of the eye- result is “positive.”
brows, smiling, and strong puffing out of the Children begin to clearly localize sound at
cheeks. The face at rest can also be examined to about 6 months. Use a noisy toy or loud voice
evaluate for any asymmetry such as widening of while observing patient reaction to evaluate
the palpebral fissure or flattening of the nasola- hearing. In younger infants and newborns, a
bial fold which might suggest weakness, keeping startle response or eye blink to loud or sudden
in mind of course that some slight asymmetries sounds can evaluate for basic hearing ability.
may be normal. This reflex is present as early as 28 weeks.
In infants and neonates, evaluating for facial Vestibular dysfunction can manifest with diverse
symmetry at rest and while crying or smiling is symptoms such as vertigo, nystagmus, emesis,
usually sufficient. Taste in older children can be and ataxic movements. Direct tests of vestibular
tested by using a concentrated solution of salt or function are beyond the scope of the basic neuro-
sugar, placed on the tongue with a cotton swab, logical exam and this chapter.
and should be reported before putting the tongue
back in the mouth to avoid detection with cranial IX and X: The Glossopharyngeal Nerve
nerve IX. Testing taste is usually done in order to and Vagus Nerves
help determine if a facial nerve deficit is due to The glossopharyngeal nerve and vagus nerves are
problems with the nerve (i.e., a “peripheral 7th” often tested together. The glossopharyngeal
such as due to a Bell’s palsy, in which case taste nerve mediates taste and sensation to the phar-
will be impaired) from a “central 7th,” where ynx, and the vagus nerve is responsible for many
taste would be expected to be preserved. functions, among which is pharyngeal constric-
tion, palate elevation, and vocal cord movement.
VIII: The Vestibulocochlear Nerve Symmetric palate elevation while saying “ahh”
The vestibulocochlear nerve mediates hearing tests the integrity of the vagus nerve, while the
and vestibular function. Finger rubs or whispers gag reflex tests both the sensory function of the
can test for hearing acuity. If a deficit is observed, glossopharyngeal nerve and motor aspects of the
use a tuning fork (256 Hz) to perform the Weber vagus. Normal swallowing suggests that these
and Rinne tests. In the Weber test, the tuning fork nerves are intact, as they must work together for
is placed at the vertex of the head. The sound the proper coordination needed to swallow.
should appear to come from the midline if hear-
ing in intact. However, if one ear is abnormal, the XI: The Spinal Accessory Nerve
sound will lateralize to the side of decreased The spinal accessory nerve innervates the sterno-
hearing in cases of conductive hearing loss (i.e., a cleidomastoid and the trapezius muscles. Head
problem with the outer ear, eardrum, or ossicles) turning against resistance tests the sternocleido-
but will lateralize to the normal ear in cases of mastoid, and a strong shoulder shrug tests the
18 A.S. May and S.T. Keros

trapezius muscles. In newborns, observe for adventitious movements such as tremors, tics,
proper neck strength with turning of the head. and myoclonus.
Isolated spinal accessory nerve lesions are rare, Another component of the motor exam is tone,
however, and routine testing in infants is rarely which describes the basic resting resistance of a
helpful unless there are other neurologic signs. muscle or group of muscles.
Tone is best examined by assessing resistance
XII: The Hypoglossal Nerve to passive movement with the subject at rest.
The hypoglossal nerve innervates the tongue, and Low tone or hypotonia can be focal, axial (i.e.,
a deficit will produce deviation to the abnormal central or truncal), appendicular (in the limbs), or
side upon tongue protrusion. Tongue strength can generalized. Increased tone or hypertonia is gen-
also be tested by asking the child to move the erally either focal or generalized.
tongue from side to side and also to push against Hypertonia can be classified as spastic, where
the interior of the cheek with resistance applied the greater the velocity of passive movement the
from the outside the mouth. In any case of sus- higher the observed tone, or rigid, where the tone
pected nerve or muscle disease, or in infants with is uniform and not velocity dependent.
generalized weakness (e.g., in suspected spinal The assessment of tone can be somewhat sub-
muscular atrophy), the tongue should be exam- jective and can be influenced by the state of the
ined at rest while in the mouth for fasciculations. patient. A resting or sleeping child can have
Tongue fasciculation can be an early indicator of lower tone than while awake. In general, the nor-
neuromuscular disease. Be careful, however, as a mal flexed posture of a newborn and relatively
protruding tongue will often have a normal increased tone decreases with age until about
tremor or movement that can be mistaken for fas- 6 months and then plateaus.
ciculations. In newborns the sucking reflex can In newborns, there are certain generalities
be utilized to evaluate lingual tone and strength. which can help to describe or indicate abnormal
tone. For example, when pulling a newborn’s arm
gently across the chest toward the opposite shoul-
Motor Examination der, there should be increasing resistance felt as
the elbow approaches the midline. If the elbow
There are several elements to the motor or crosses the midline without excessive force, this is
strength portion of the neurological exam. First, a an indication of decreased tone, and this is some-
simple observation of positioning at rest may times referred to as a positive “scarf sign.” In
show atypical postures or asymmetries which infants, axial tone can be evaluated by holding the
may suggest weakness or abnormalities of tone. patient in a ventral suspension and observing the
The preterm infant, for example, keeps the limbs position of the child draped over the arm. Infants
in an extended position but the normal full-term with normal tone should make some attempt to lift
infant has flexed extremities at rest. Some char- the head and keep the back arched. Additionally,
acteristic poses, such as “frog-leg posturing,” or one can raise the infant from supine to sitting by
external rotation of the legs suggests a systemic pulling on the arms and observing for any abnor-
weakness. Scissoring of the legs, where the legs mal head lag (no lag by 6 months of age), a sign of
tend to cross at the feet or ankles, is one sign of axial hypotonia and neck weakness.
possibly increased tone of the hip adductors and Formal strength testing usually involves test-
is often seen in children with spasticity from ing for strength in an isolated muscle group. Both
upper motor neuron injuries such as corticospinal muscle bulk and strength should be graded. The
track injuries. Fisting of the hand or holding the most common grading system ranges from 0 to 5,
thumb adducted across the palm is a position where zero corresponds to no movement, 1 repre-
which also suggests corticospinal tract involve- sents a flicker of movement or muscle contraction
ment. Observation at rest will also usually reveal which does not result in limb motion, 2 indicates
2 The Neurologic Exam in Neonates and Toddlers 19

movement but not against gravity (i.e., in the Proprioception, or joint position sense, can be
plane of the bed), 3 is movement against gravity tested by holding the sides of a joint (e.g., the lat-
but without resistance, 4 is movement against eral aspects of the big toe) and moving the distal
resistance, and 5 is full power or movement aspect of the joint gently and briefly in an up or
against strong external resistance. down direction. A healthy person should be able
In infants and young children, strength testing to detect even very small movements and specify
is typically performed while observing functional the direction. Vibration sense, which is transmit-
movements. For example, symmetric and age ted together with proprioception in the dorsal col-
appropriate reaching, crawling, or cruising sug- umn system of the spinal cord, is best tested by
gest normal strength. Of note, asymmetry in placing a 128 Hz tuning fork on a bony portion of
reaching or demonstration of a hand preference a finger, toe, or other joint.
before about 1 year of age may be a sign of patho- Sensory testing in newborns and infants is
logic weakness in the non-preferred arm or hand. usually limited to basic testing while assessing
for motor output as a potential response to a stim-
ulus. For example, an infant will usually with-
Sensory Examination draw a limb in response to a tuning fork. This
procedure also provides temperature and touch
The sensory examination includes evaluation of stimulation and trial-to-trial variability in infant
the primary modalities of pain, temperature, testing is common. Noxious stimuli such as nail
touch, proprioception, and vibration sense. bed pressure, pinching of the skin, or pinprick
Formal sensory testing can be done typically after should also elicit a cry or withdrawal. Light touch
5–6 years of age. A complete sensory examina- or tickling will also usually precipitate a with-
tion can take a long time to perform in a healthy drawal of the foot, for example. Again, a sensory
subject. In reality, the sensory exam is usually tai- exam is most sensitive when there is a specific
lored to a specific area of concern where a deficit area of concern for neurologic injury.
is already suspected, as it will seldom reveal an
abnormality which has not been previously self-
reported. Pain sensation can be tested with a ster- Reflex Examination
ilized pin, being careful to apply the same pressure
in all areas. However, it is quite difficult to apply In neurology, reflexes can refer to either the deep
consistent, nontraumatic pressure with each tendon reflexes mediated by stretch receptors in
application. Furthermore, certain areas of the skin the joints and muscles or to developmental and
are naturally more sensitive than others. Thus, behavioral reflexes. Deep tendon reflexes test the
variations in the exam are to be expected, and sensory integrity of the stretch receptors and
mild sensory deficits or asymmetries should be associated sensory nerves and also require a
treated suspiciously and placed in context of the functioning muscle to produce the motor
overall history and other examination findings. In response. Most reflexes are mediated by only one
general, asking the subject if the pin feels sharp is or two spinal nerve roots and are very helpful
acceptable, as opposed to attempting to quantify when attempting to localize an injury. Upper
the degree of pain or sharpness. Temperature sen- motor neuron injuries (those arising in the cortex
sation is transmitted together with pain by the spi- of corticospinal track) will lead to a condition
nothalamic tract and can be tested by placing a where deep tendon reflexes (and overall tone) are
cool metal object against the skin. Touch is evalu- elevated, while lower motor neuron injuries (i.e.,
ated by using a cotton swab or the examiners fin- the cell bodies of the motor neurons in the spinal
ger and gently pressing, not wiping, against cord and their associated nerve fibers) or muscu-
the skin. With eyes closed, the subject should be lar injury will cause depressed or absent reflexes.
able to identify the location of the stimulus. When testing the tendon reflexes at a joint such
20 A.S. May and S.T. Keros

as the elbow or knee, the joint should be relaxed and then allow the examiner to classify the
and bent approximately 90°. A brisk tap with a reflexes as abnormal or normal depending on the
reflex hammer provides the stretch response and rest of the exam.
the reaction is a contraction of the muscle. The There are also some reflexes known as “non-
magnitude of tendon reflexes is dependent on the stretch” or superficial reflexes. Among these is
location as well as strength of the tap with the the abdominal reflex which is elicited by stroking
hammer. Tendon reflexes are most commonly or lightly scratching one side of the abdomen.
tested in the arms at the biceps (mediated by C5 This should trigger a small ipsilateral contraction
and C6) just anterior to the elbow, the brachiora- of the abdominal muscles and is one way to test
dialis (also C5/6) above the wrist on the radial for sensation and motor integrity of the thoracic
aspect of the forearm, and the triceps (C6/7) just roots 8 through 12, which may be damaged in a
posterior to the elbow. In the legs these include spinal cord injury, but does not otherwise have
the patellar reflex (L2/L3/L4) elicited by a strike large associated muscle groups for easy testing.
just below the patella and the ankle reflex (L5/S1/ The cremasteric reflex causes elevation of the
S2) produced by tapping the Achilles tendon, ipsilateral testicle upon upward stimulation of the
usually while maximally flexed. The adductor inner thigh. This reflex is mediated by L1 and L2.
reflex (L2–4) is tested by tapping the adductor Anal tone and the anal “wink” reflex are medi-
tendon near the medial epicondyle of the distal ated by S2–S4.
femur. The most common grading system ranges One of the most widely tested superficial
from 0 to “4+” where 0 refers to no reflex, 1+ is a reflexes is the plantar reflex.
weak reflex, 2+ is a normal reflex, 3+ is hyperac- In children aged 1 or older, a normal plantar
tive, and 4+ generally indicates some clonus or reflex produces a plantar flexion of the large toe,
spreading. A “crossed adductor response” is one while an upward or extensor response is consid-
of the more common examples of a reflex spread- ered abnormal. An extensor response of the toe in
ing and occurs when a tap of one thigh adductor non-infant is evidence that this otherwise spi-
leads to bilateral adductor muscle contraction. nally mediated reflex is not suppressed by infor-
Clonus is most easily assessed at the ankle, elic- mation from the cortex.
ited with a rapid jerk of the ankle into a dorsi- Thus, an extensor response is another sign of
flexed position. If present, clonus will appear as a upper motor neuron injury. There are nearly a
repeated movement or beating of the dorsiflexed dozen methods which can be used to test for the
foot into plantar flexion. Some beats of clonus plantar response, but the most often used is
can be normal in newborns and young infants as Babinski test. The Babinski test involves stroking
well as teenagers and some adults, but many the lateral side of the bottom of the foot up toward
beats of clonus or sustained clonus should prompt the ball of the foot and then curving in toward the
a more thorough examination of possible upper toes. An initial upward movement of the big toe
motor nerve injury. is considered a “positive” Babinski sign. A down-
Although deep tendon reflexes can be tested at ward movement or no movement is considered
any age, the triceps reflex is particularly difficult the normal situation. The plantar reflex is medi-
to elicit in neonates. Tendon reflexes can also be ated by the L4-S2 spinal roots. In some cases, a
difficult to achieve in babies because of their con- plantar or extensor response is unequivocal.
stant motion and light weight, and thus nonspe- However, testing can be variable from trial to
cific movements as well as movement due to the trial, particularly because it is sometimes difficult
hammer itself can be interpreted as a reflex not to elicit a tickle or withdrawal response upon
response. In addition, their small size makes for a testing. Note that infants will have a normally
small target; the biceps tendon and patellar ten- positive Babinski sign, as a result of an immature
don require a precise hit from the hammer, which brain. However, a lack of a Babinski sign in an
can take practice or repeated trials. With children, otherwise healthy infant does not have particular
it is often sufficient to simply use “low” or “brisk” neurologic significance.
2 The Neurologic Exam in Neonates and Toddlers 21

Developmental Reflexes and move it forward, as if taking a step. The


Landau response is triggered with ventral sus-
These are the final group of reflexes which are pension of the child or placement in the prone
commonly tested as part of the neurologic exam. position, which should result in an extension of
These are reflexes typically present early in life both the head and legs. This reflex begins around
that extinguish with progressive maturation of 3 months and persists through infancy. The tonic
cortical inhibitory function. These reflexes have neck response describes a flexion of the limbs of
two useful functions in neurology. First, their the contralateral side and extension of the limbs
persistence past a certain age may be a clue of a on the ipsilateral side in response to a force head
systemic problem. Secondly, and somewhat more turn. This usually diminishes by 4–5 months,
commonly, is that these reflexes can be used to which is the age many babies begin to roll over.
elicit a motor response which an infant would Of note, a normal tonic neck reflex can provoke
otherwise not do voluntarily. asymmetries in tone and position, and thus it is
For example, the Moro reflex can be valuable important to make sure the head is midline during
in trying to determine whether there is asymmet- a neurologic examination. Refer to Table 2.1 for
ric weakness or injury such as might occur in a a list of some developmental reflexes and their
clavicular fracture or perhaps a brachial plexus expected age resolution.
injury during birth. The Moro reflex refers to the
abduction and subsequent adduction of the arms
elicited by a sudden loss of head or truck support, Coordination
which creates a sensation of falling. This reflex is
readily elicited in a newborn and disappears by 5 Although coordination is commonly thought of
or so months of age. A grasp response is the well- as cerebellar-mediated process, coordination is
known curling of the fingers or toes when a finger the integration of many different pathways which
or other object is placed in the palm of the hands are all necessary to produce accurate movements.
or against the balls or soles of the feet. The suck- As a result, impaired coordination can be seen in
ing reflex and rooting reflexes are also examples disorders of the cerebellum and basal ganglia as
of common developmental reflexes and can be well as various other sensory and motor systems.
used to stimulate motor activity for neurologic Finger-to-nose testing is an easy way to assess
testing. The stepping reflex is present during coordination and simply requires the subject to
the first 6 weeks of life. This reflex is stimulated move his or her finger from the nose to an exam-
by holding an infant upright and resting the iner’s finger or other object, which should be
feet against a surface with the knees slightly bent. placed as far from the subject while still being
The typical response is for the infant to raise a leg reachable, as this distance adds some sensitivity

Table 2.1 Developmental reflexes and their expected age of resolution


Reflex Stimulus Response Resolution
Rooting Stroke cheek or lip Head turns and mouth opens 2–3 months
toward stimulus
Stepping Hold upright with feet touching surface Moves feet as if taking steps 3–4 months
Tonic neck Placed on back, turn head to side Ipsilateral arm extension 4–5 months
and contralateral flexion
Moro Sudden falling motion or loud noise Arms extend and abduct, 5–6 months
then adduct
Palmar grasp Press palm surface Finger curl 5–6 months
Plantar grasp Press sole of foot Toes curling 9–10 months
Landau response Place on stomach Back arches and head raises Starts at 3 months,
resolves at 12 months
22 A.S. May and S.T. Keros

to the test. The subject should be asked to go nervous system. To some degree, if a child can
from finger to nose and back somewhat briskly, successfully walk or run down a hallway, stop,
but excessive speed is not necessary to evaluate and return when asked, the majority of the neuro-
coordination. Rather, the subject should be able logic exam is surely normal, as they have demon-
to accurately and very precisely touch the finger- strated relatively intact hearing, cognitive, motor,
tip to the examiner’s fingertip. During this test, visual, and sensory pathways. It is best to evalu-
observe for any dysmetria, which can take the ate gait by observing the child while barefoot.
form of missing the target laterally or “pass Note the station or the stance of the patient, their
pointing.” Beware of any vision abnormality or steps, the speed, and the steadiness of the gait.
impaired depth perception which will confound Steadiness varies with age and should be consid-
the results. Finger-to-nose testing is also a good ered in the context of the patient’s age.
way to evaluate for an intention tremor. An anal- Cooperative children should be asked to walk
ogous test in the lower extremities involves hav- regularly, then on their toes and heels, and finally
ing the patient place the heel of one foot on the with one foot directly in front of the other. This
knee of the opposing leg and carefully sliding the ability to tandem walk is usually present by 5
heel down the shin toward the ankle and back years of age. For many abnormalities, the gait
again. Another test of coordination is the rapid will be affected in predictable or stereotypical
alternating movements test, which when abnor- ways. For example, focal weakness in one leg
mal is called dysdiadokinesis. One way to per- may make toe walking difficult on that foot or
form this test is to have the subject rapidly tap the result in a limp or other noted asymmetry. Truncal
index finger to the thumb or alternately to tap a ataxia or ataxia of a leg will result in a state of
thigh or other surface with the dorsum of the imbalance and appear clumsy and staggered,
hand and then the palm of the hand as quickly as with a wide-based gait. Arm weakness or tone
possible. Rapid foot tapping is an analogous test may be seen as a decreased arm swing. Other
in the lower extremities. Note that mild weakness characteristic gait abnormalities are somewhat
will tend to slow any rapid alternating move- less intuitive to use in pinpointing the abnormal-
ments, and thus this is a good test for evaluating ity. Circumduction, for example, is a lateral
subtle unilateral weakness in a hand or foot, by swinging movement of the foot with excessive
comparing speed of movements to the other side. lifting at the hip while bringing the foot to the
Testing coordination is not feasible in new- front, which is seen in cases of foot drop or distal
borns and infants, due to lack of established cir- weakness. A spastic gait due to increased tone,
cuitry, in addition to the obvious inability to such as might be seen in cerebral palsy, will
follow commands. By several months of age, appear shuffling, with the legs adducted and the
however, simply testing for abnormal movements child somewhat on the toes or front of the foot.
upon reaching for objects will give some sense of An antalgic gait describes the traditional limping
coordination and must of course be compared to pattern seen with maneuvers to limit bearing
the abilities of other children at that age. Midline weight on a painful leg.
or truncal ataxia, which can arise from lesions in Obviously, gait can only be tested once a child
the vermis of the cerebellum such as might be begins walking. However, an evaluation of crawl-
seen in a meduloblastoma, will affect the ability ing, standing, or cruising can be relatively effec-
to sit normally or will affect the balance of a tive proxies for the information provided by
walking child. independent walking.

Gait Conclusion

A gait exam is an essential part of any neurologi- The neurologic examination as presented in this
cal examination, and a normal gait requires the chapter is intended to be a brief reminder or sum-
normal functioning of all components of the mary of various ways to identify and describe
2 The Neurologic Exam in Neonates and Toddlers 23

suspected neurologic abnormalities in children. • Understanding and interpreting the neurologic


There is of course tremendous variation among exam in children requires in-depth knowledge
clinicians in their overall approach and examin- of normal development.
ing techniques. However, the examination and its • Handedness in infants under 1 year of age
components as described represent a solid frame- may indicate a relative weakness or neuro-
work which should help any pediatric health-care logic problem in the nondominant side.
provider when trying to determine whether a par-
ticular set of complaints, symptoms, or signs are
abnormal and whether they may warrant addi- Suggested Reading1
tional work-up and neurological or neurosurgical
consultation. Aminoff MJ, Simon RR, Greenberg D. Clinical neurol-
ogy. Europe: McGraw-Hill Education; 2015.
Fenichel GM. Clinical pediatric neurology: a signs and
symptoms approach. 3rd ed. Philadelphia: WB
Clinical Pearls and Red Flags Saunders; 1997.
Newman SA, Ropper AH, Brown RH. Adams and Victor’s
principles of neurology. 8th ed. New York: McGraw-
• While a systematic and complete neurologic
Hill Medical; 2005.
exam is occasionally needed, observation
alone of a child at rest and play can elucidate
a tremendous amount about both normal and
abnormal neurologic findings.
• To some degree, if a child can successfully
walk or run down a hallway, stop, and return
when asked, the majority of the neurologic 1
There are many excellent basic neurology textbooks
exam is surely normal, as they have demon-
which contain extensive discussions about the various
strated relatively intact hearing, cognitive, aspects of the neurologic exam presented in this chapter.
motor, visual, and sensory pathways. Several are listed here in lieu of a specific reference list.
http://www.springer.com/978-1-4939-3805-6

You might also like