Head

Examination of the head must be done systematically, looking for asymmetry, indentations, and protuberances. Evaluation of the fontanels and cranial sutures should be performed with gentle palpation. The dimensions of the anterior fontanel should be carefully recorded [Pedroso et al., 2008]. The examiner should determine by observation and palpation the presence of frontal bossing, bulging fontanel, sutural synostosis or diastasis (separation), and unusual head shapes such as trigonocephaly, marked dolichocephaly or brachycephaly. Positional plagiocephaly has become increasingly common with the current “back to sleep” approach, and is the most common cause of abnormal head shape; it can often be distinguished from isolated craniostenosis by prominence of the contralateral forehead, which leads to a rhomboidal appearance [Bialocerkowski et al., 2008]. Unusual masses under the scalp and gross asymmetries of the skull should be sought.

The occipitofrontal circumference should always be measured. If the child becomes agitated, the measurement could be deferred until later in the examination (stage 3). The largest measured circumference incorporating nasion and inion should be recorded and plotted on a standardized graph of normative data, preferably incorporating serial measurements. The size of the anterior fontanel, which is typically closed by 12 months of age, should be recorded. The anterior fontanel pulsates in unison with the heartbeat, and becomes fuller or bulging when the child cries, and this must be distinguished from disease states in which there is increased intracranial pressure. The tenseness of the anterior fontanel should be evaluated when the child is sitting comfortably in an upright position. The posterior fontanel usually admits only a finger at time of birth and is usually closed by 2 months of age. Other fontanels are usually difficult to palpate, except in pathologic states. Occasionally, accessory fontanels may be found along the sutures, particularly the sagittal suture; these are usually benign variants. The head should be auscultated for the presence of unusual intracranial bruits. Intracranial bruits occur commonly in childhood, and cautious interpretation is advised; asymmetric bruits and those that can be suppressed by carotid artery suppression are frequently pathologic. Vascular abnormalities, such as vein of Galen malformations, can produce extremely loud bruits, and are often heralded by prominent scalp veins. The vein of Galen malformation is typically associated with increasing occipitofrontal circumference, high-output cardiac failure, and infant distress while in the supine position.

Cranial Nerves

Most of the examination of cranial nerve function of the infant and toddler can be completed by observation with minimal invasive procedures. More details concerning examination of each cranial nerve can be found in Chapter 2. Toys or colorful objects can facilitate the assessment of extraocular movements in young children. Visual fixation and pursuit will bring out nystagmus and strabismus. If the child appears uninterested in bright objects, the possibility of a visual defect or an underlying intellectual defect must be considered. Rolling eye movements and dysconjugate gaze suggest gross visual impairment. Double simultaneous stimulation (i.e., simultaneously bringing two bright objects into both temporal fields) normally causes the child to look from one object to the other; failure to take notice of one object may indicate homonymous hemianopsia. An opticokinetic tape (with repetitive bars or objects) should be drawn horizontally and then vertically across the child’s field of vision. An absent response results from lack of visual fixation or from gross impairment of vision. Unusual transient deviations of the eyes may occur in the first year of life [Echenne and Rivier, 1992].

A beam from a small flashlight should be directed at each eye to allow evaluation of pupil size (noting colobomas and anisocoria), pupillary responses and the red retinal reflex. To avoid frightening the child, the examiner could use the light in a playful manner such as directing the light beam at the child’s hand or abdomen while trying to avoid restraining the child or forcibly opening the eyes. There are many eye features to be noted, including symmetry of the palpebral fissures, relative size of the two globes, angulation of the eyes compared with other facial components (i.e., mongoloid or antimongoloid slant) and with the ears, cataracts, conjunctival telangiectases, colobomas of the iris, ptosis, proptosis, and malformed or eccentrically placed pupils. Hair color, patterning distribution, including unusual whorl patterns, and texture should be assessed.

The examiner should observe the child’s facial movements closely throughout the entire examination. Smiling at the child, tickling the child, or making unusual noises or facial grimaces often causes the child to smile or laugh, allowing observation of the nasolabial folds. Widening of the ipsilateral palpebral fissure or inability to bury the limbus when crying is indicative of facial nerve weakness. If facial weakness is found, one must distinguish central from peripheral cranial nerve VII dysfunction. The latter includes involvement of the muscle that raises the ipsilateral eyebrow. In the younger infant, sucking and rooting reflexes should be obtained. Tongue thrusting, drooling, and unusual shapes of the lips should be evident. Sometimes, the child can be induced to protrude the tongue if the examiner urges the child to imitate the examiner’s tongue movements. Deformity, atrophy, or abnormal positioning of the tongue can be observed. Tongue fasciculations should be evaluated with the tongue in the resting position, and by gently elevating the tongue with a depressor and examining the undersurface.

Basic responses to the sound made by a tuning fork, rubbing fingers together, ringing a bell, or using a toy noisemaker that generates noise at a modest volume may provide much information. The examiner must be careful not to confuse response to a visual cue (e.g., the movement needed to elicit noise from a toy) with response to the sound. Typically, the young infant orients toward the sound by turning the head with arrest of motor activity, while the child older than 6 months usually reaches for the noisemaker. If the child fails to perform satisfactorily, formal audiometry and auditory-evoked response testing should be obtained.

Motor Evaluation

As with all other parts of the evaluation, the motor examination begins with observation. Even before touching the child, the examiner should systematically observe the general posture and the symmetry of movements of arms and legs, and look for any gross discrepancies in muscle bulk or limb length. Definite hand preference (such as reaching across the midline to avoid using the contralateral hand) before 24 months suggests a central or peripheral nervous system impairment of the opposite hand and arm. In this situation, leg movement and use should be carefully studied to detect the presence of hemiparesis. Typically, there is also a decrease in spontaneous movement of the affected limb. Unusual posturing of the limbs may indicate paresis or evolving extrapyramidal disease.

Decreased muscle bulk may not be appreciated because of the large amount of subcutaneous fat, and muscle atrophy may be undetected. The examiner should be careful to palpate muscle mass beneath the fat and not to misinterpret the subcutaneous tissue as muscle.

Palpation is the next step; this allows for evaluation of muscle tone, the resistance of muscle to passive stretch. Muscle tone and range of motion of the arms and legs are best assessed when the child is in the relaxed state by gently shaking and moving the hands and feet in flexion and extension. Pronation and supination of the hands and forearms provide further information about range of motion and the presence of spasticity or rigidity. Greater than normal resistance to passive movement indicates hypertonia, whereas less than normal resistance indicates hypotonia. This portion of the examination is most difficult for the novice because reasonable experience is required to make accurate judgments. It is important to distinguish increased tone from limitation of movement due to joint contracture. Spontaneous muscle movements, particularly those against gravity, provide the most information concerning muscle strength. Active resistance to attempted movement of the extremities will provide the examiner with an estimate of muscle strength.

Upper motor neuron unit involvement, such as that in hemiparesis, may manifest by decreased movement of the entire extremity or more specific involvement such as limited flexion of the arm at the elbow, persistent fisting, or adduction of the thumb against the palm. Children with Erb’s brachial plexus injury commonly hold the arm against the chest in a position of internal rotation and adduction at the shoulder. Interacting with the infant using toys and other interesting objects may facilitate the evaluation of limb strength, range of motion, and coordination. In the older cooperative child, individual muscle testing should be carried out when appropriate.

There is a normal developmental sequence of fine motor control as the child becomes more adept at reaching for objects. Grasping things with both hands and holding the object before the face or immediately placing it in the mouth is later superseded by transferring the object from hand to hand and manipulating the toy. The infant’s grasping skills are best demonstrated in the response to small objects. The 4–5-month-old infant is able to grasp an object with the entire hand (Figure 3-1); at 7 months the thumb and the neighboring two fingers are used (Figure 3-2); and the pincer grasp (using only the thumb and forefinger) should be present by 9–11 months (Figure 3-3). The palmar grasp reflex (i.e., obligate grasp reflex) should gradually diminish from 3–6 months of age. Teleologically, this allows the infant to develop the ability to transfer objects from hand to hand. The persistence of the obligate grasp reflex beyond 6 months of age may signal corticospinal tract dysfunction. Observation of the child’s ability to raise the arms and to abduct and adduct the arms while reaching for a proffered object provides valuable information concerning proximal muscle strength. Simultaneously, the presence of intention tremor and of the avoidance response of early athetosis may be evident. Congenital malformations of the fingers and hands from webbing to clinodactyly can be readily determined during this portion of the examination.

Fig. 3-1 Entire hand grasp of a 4-month-old infant.

(Courtesy of the Division of Pediatric Neurology, University of Minnesota Medical School.)

Fig. 3-2 Use of two fingers and thumb in the grasp of a 7-month-old infant.

(Courtesy of the Division of Pediatric Neurology, University of Minnesota Medical School.)

Fig. 3-3 Pincer grasp with the thumb and forefinger of an 11-month-old infant.

(Courtesy of the Division of Pediatric Neurology, University of Minnesota Medical School.)

Direct examination of the hips should include assessment of the range of motion; decreased excursion may signify spasticity or subluxation of the hip joints. Subluxation may exist separately or as a result of spasticity. Conversely, increased excursion may represent hypotonia or ligamental laxity.

Initial examination of the legs should consist of assessment of muscle symmetry and mass. The presence and symmetry of spontaneous motor movements should be evaluated. Assessment of tone is similar to that done with the arms and hands – one should gently shake the feet and passively move the joints of the lower extremities from hip to knee to ankle. Tight Achilles tendon is shown by decreased range of motion and inability of the feet to dorsiflex readily beyond neutral (90 degrees).

Deep tendon reflexes that are excessively brisk may indicate upper motor neuron unit disease, especially when associated with clonus. Asymmetry is particularly worrisome because of the association with pathologic conditions. Absent deep tendon reflexes are seen with anterior horn cell disease or peripheral neuropathy. The crossed adductor reflex is elicited when the patellar reflex is stimulated and resultant contraction of the adductor muscles occurs in the opposite leg. This response can be normal until approximately 1 year of age. However, persistence of the response, particularly unilaterally, suggests the presence of corticospinal tract involvement.

The plantar response can be as important in infants as in adults. There is no consensus of opinion about the latest time at which an extensor response is a normal finding. One report found an extensor response in 50–75 percent of 1-year-old infants [Dodge, 1964]. Others claimed that the usual response in the newborn period is flexor in origin and that the initial response is flexor in 93 percent of normal infants [Hogan and Milligan, 1971]. All would agree that asymmetric extensor toe signs or extensor toe signs that persist beyond 12 months should be considered pathologic. A pathologic extensor plantar sign is indicative of upper motor neuron unit disease. Unsustained ankle clonus up to six beats is often present in the neonatal period. Ankle clonus should disappear by 2 months of age. The persistence of ankle clonus and extensor plantar response suggests upper motor neuron unit disease even in the absence of hyperreflexia.

Cerebellar function is difficult to assess in infants; assessment is easiest in a cooperative child capable of sitting, standing, walking, or reaching for objects. The examiner can also observe the child during play to see resting or intention tremor, dysmetria, titubation or truncal sway while sitting, and fine motor coordination. Decreased tone may accompany other signs of cerebellar dysfunction.

Sensory Testing and Cutaneous Examination

Light touch can be tested by gently stroking the extremities; this should lead to a reaction with signs of recognition ranging from eye deviation and facial response to anxious withdrawal of the limbs (Figure 3-4). Application of a tuning fork often causes arrest of motion and a wide-eyed look of wonder in the child who cannot otherwise describe the feeling. Proprioception cannot be directly evaluated at this age, but observations of sitting positions, gait, and posture may provide some clues. Pain response from light application of a pin or gentle pinching should be reserved until late in the examination. The child may cry or make short, whimpering sounds. Careless use of the pin can destroy rapport with the patient and loss of confidence on the part of the caregivers.

Fig. 3-4 Segmental distribution of the cutaneous nerves of an infant.

(Modified from Fanaroff AA, Martin RJ. Neonatal-perinatal medicine: diseases of the fetus and infant, 5th edn. St. Louis: Mosby, 1992.)

The skin of the infant is observed for obvious areas of abnormality that may suggest certain conditions, including neuroectodermal neurocutaneous disorders. Examination of the spine may indicate the presence of scoliosis, sinus tracts, scars, dimples, and hemangiomas. Unusual skin lesions or hair growth over the spine suggest the presence of an underlying mesodermal defect, including diastematomyelia. The spine should be palpated along its entire course for defects.

Abdominal and cremasteric reflexes are present at birth. The abdominal reflex is elicited by stroking the skin of the upper, middle, and lower portions of the abdomen laterally from the midline. Each stroke elicits a muscle contraction mediated by a different group of thoracic nerves from T8 to T12. The response results in the retraction of the umbilicus toward the stimulated side. The cremasteric reflex is elicited by upwardly stroking the inner thigh, beginning 3–5 cm below the inguinal crease. The cremasteric reflex results in an elevation of the testicles due to contraction of the overlying smooth muscles. Cremasteric reflexes are mediated by spinal nerves L1–L2.

Motor Performance Instruments

Through the years, several instruments have been devised that are useful for evaluating motor performance in relation to chronologic age. These instruments have provided norms for evaluating the expected rate of motor development for a number of different assessments and maneuvers [Zafeiriou, 2004]. The instruments that are most commonly used are listed in Box 3-2.

Box 3-2 Most Commonly Used Motor Performance Tools

Developmental scales

Amiel-Tison [Amiel-Tison, 1968]

Amiel-Tison [Amiel-Tison, 2002]

Bayley [Bayley, 1969]

Brazelton [Brazelton and Nugent, 1995]

Dubowitz [Dubowitz et al., 1999]

Haataja [Haataja et al., 1999]

Peabody [Reed, 1985]

Prechtl [Prechtl and Beintema, 1964]

Developmental screening tests

Denver Developmental Screening Test (Denver II) [Frankenburg et al., 1992]

Battelle Screening Test [Glascoe and Byrne, 1993]

Clinical Adaptive Test (CAT)/Clinical Linguistic and Auditory Milestone Scale (CLAMS) [Leppert et al., 1998]

Knobloch Revised Screening Inventory [Knobloch et al., 1980]

General Movements (GMs) Assessment [Dargassies, 1997; Prechtl, 1997; Prechtl et al., 1997]

Motor assessment instruments

Alberta Infant Motor Scale (AIMS) [Piper et al., 1992]

Early Motor Pattern Profile (EMPP) [Morgan, 1988]

Gross Motor Function Measure (GMFM) [Russell et al., 2002]

Movement Assessment Inventory (MAI) [Kaye and Whitfield, 1988]

Test of Infant Motor Performance (TIMP) [Campbell et al., 1993]

(Adapted from Zafeiriou DI. Primitive reflexes and postural reactions in the neurodevelopmental examination. Pediatr Neurol 2004;31:1.)

Developmental Reflexes

Developmental reflexes are patterned responses that are ontogenetically determined at certain ages. They represent maturational stages of the developing nervous system, and therefore they can be helpful in assessing neurologic development. Occasionally, they can have localizing value, but usually, they are non-specific. Abnormal findings include the absence or poor manifestation of the expected response, persistence of a reflex that should have disappeared, or an asymmetric response. These reflexes and the means of elicitation are listed in Table 3-1 and Table 3-2 [Zafeiriou, 2004].

Table 3-2 Eliciting Postural Reactions

(Data from Vojta, 1988; Zafeiriou et al., 1998; Zafeiriou, 2004.)

The Moro reflex presents in an incomplete fashion with an attenuated adduction phase at 2 months, and is seen in its complete fashion until 5 or 6 months of age. Although the Moro reflex may be demonstrated by different maneuvers, correctly eliciting it requires holding the infant in the supine position, lifting the head and then allowing it to fall approximately 30 degrees while cradling the head in the examiner’s hands [Parmelee, 1964]. The expected response is initial extension and abduction of the arms with extension of the fingers, followed by adduction of the arms at the shoulder (Figure 3-6). An abnormal Moro reflex usually represents diffuse central nervous system depression, generalized weakness, or severe spasticity rather than a specific area of involvement. An asymmetric Moro reflex can be caused by unilateral brachial plexus palsy, fractures of the humerus or clavicle, or spastic hemiplegia. An exaggerated Moro reflex may indicate pathologic central nervous system (CNS) irritability. The Moro reflex can be distinguised from similarly presenting extensor infantile spasms since the normal developmental reflex is always associated with a postural change and never occurs spontaneously or in clusters.

Fig. 3-6 The Moro response to rapid extension of the neck in a 2-day-old infant.

The abduction phase of arm movement is illustrated. A cry usually accompanies the response, and the leg position varies.

The asymmetric tonic neck reflex (ATNR) may be detected in the neonatal period but reaches its peak at 2 months. It gradually diminishes and is absent by 6 months of age. The tonic neck reflex has been described in great detail in animals and humans [Shevell, 2009]. To elicit the reflex, the head is turned to one side while the infant is lying in the supine position. There is extension of the arm and leg on the side toward which the face is turned, while the contralateral extremities flex (“fencer’s posture”). The degree of response varies widely but is usually seen to the same degree in each direction. In any event, a normal infant should not maintain the position beyond a few seconds (i.e., obligate ATNR). This reflex should disappear by 6 months of age [Paine et al., 1964]. When the ATNR can only be elicited to one side, it may indicate a lesion in the hemisphere opposite the direction in which the face is turned. The same consideration holds true if the response is obligate or persists beyond the expected age. Severe muscle weakness secondary to central or peripheral motor unit involvement may lead to an abnormally diminished response. Athetoid and spastic infants may also have an exaggerated response, and this may contribute to their difficulty in sitting or standing.

The palmar grasp reflex is elicited by placing an object or the examiner’s finger in the palm of the infant’s hand; this leads to an involuntary flexion response. This reflex subsides by 3–6 months of age, and is replaced by voluntary grasping, which is necessary to allow transfer of objects from hand to hand. The obligatory involuntary grasp reflex may persist and is often one of the earliest signs of infantile hemiplegia [Paine et al., 1964].

In slightly older infants, the Landau reflex can be first elicited between 5 and 10 months of age, and can usually be seen up to 2 years of age. With one hand supporting the abdomen in the prone position, the examiner flexes the infant’s head with his other hand. The normal response is flexion of the legs and trunk. When held in horizontal suspension, 55 percent of infants spontaneously elevate their heads above the horizontal plane by age 5 months and 95 percent by 6 months [Paine et al., 1964].

The placing reflex response can be demonstrated by holding the upright infant in a manner that causes the dorsal surface of the infant’s feet to touch the underside of a tabletop. The infant flexes the legs at the hips and knees so that contact with the underside of the surface ceases.

One of the most useful maneuvers is the traction response. This is elicited with the infant in the supine position; the examiner grasps both hands and pulls the infant gently and slowly upward, to a sitting position. Marked head lag with little resistance to the examiner’s pulling efforts characterizes the newborn response (Figure 3-7). By 1 month, the infant’s head shows transient neck flexion followed by extension as the infant is pulled forward. Usually, by 3–5 months of age at the latest, the infant is able to participate actively with arm flexion at the elbow, as well as holding the head and trunk in a straight line as the examiner pulls him or her to the upright position. At this point there should be no head lag, and little or no forward motion of the head as the child reaches the upright position. Asymmetry signals a neurologic difficulty. Superimposition of leg extension with standing as the infant is pulled to sitting suggests bilateral corticospinal tract difficulty.

Fig. 3-7 The traction maneuver causes little response in a 2-day-old infant.

There is little or no perceptible flexion of the neck or the arms at the elbows.

A stereotypic “elbowing” movement in newborns has been described. A curved wooden model of an ultrasonographic probe is gently used to exert pressure on the right and left subcostal regions. The newborn reacts with a particular defensive arm movement in which there is a three-phase response [Saraga et al., 2007].

A valuable measure of vestibular function in the newborn can be obtained by holding the infant in a supine position with the feet closest to the examiner. As the examiner rotates the infant laterally in each direction, the eyes of the infant deviate in the direction of rotation, accompanied by intermittent nystagmus to the opposite side. This maneuver also allows extraocular movements to be assessed.

It is essential that the examiner take into account the overall pattern of developmental responses. An isolated delay or irregularity of one reflex does not indicate significant neurologic abnormality. There are other developmental reflexes, but those discussed here appear to be the most often evaluated and the most useful.