Parameters	Upper Motor Neuron Lesions: Central Nervous System Dysfunction*	Lower Motor Neuron Lesions: Peripheral Nervous System Dysfunction†
Intellect	Deficits may be found with cortical abnormalities	Normal
Cranial nerves	Abnormalities usually reflect brainstem involvement but may indicate a neuropathy	May be involved
Power	Slightly decreased, although movement more severely impaired because of altered tone	Markedly reduced; neuromuscular junction disease associated with fatigue
Tone	Increased (spasticity) with lesions affecting pyramidal pathways; rigidity seen with extrapyramidal disease	Reduced (floppy or hypotonic)
Coordination	Impaired when cerebellum or its connections are involved	May be hindered by weakness
Reflexes	Hyperactive in pyramidal dysfunction; plantar stimulation results in an extensor response (Babinski) in the great toe	Difficult to elicit
Sensation	Usually intact, but spinal cord gives a sensory level that is in a dermatomal distribution	Impaired with lesions that affect the nerve
Fasciculations		Present with anterior horn cell disease but occasionally also found with neuropathies

* Involve intracranial contents, brainstem, or spinal cord.

† Involve intracranial horn cells, nerves, neuromuscular junction, or muscles.

Obtaining the History

When combined with a hands-off observation, the history usually provides the diagnosis (see Chapter 1). The physical examination rarely reveals previously unsuspected findings.

Key Point

The history usually provides the diagnosis.

The school-aged child

After introducing myself to the children’s families, I try to establish a rapport with the children by asking why they think they have come visit me and what they think is wrong. Ask children their names and what they like to be called. Some children prefer to be called by a nickname.

Key Point

Never intimidate the child.

Start the history by talking directly to the patient. This helps the child feel more involved in the process. Begin by talking about nonthreatening subjects, such as family members, school or teachers, television shows, music, or sports. Use open-ended questions to avoid “Yes” or “No” answers. All school-aged children deserve the opportunity to speak to the physician alone; it is during this part of the history that they sometimes reveal information they would otherwise withhold. The parents must be asked to leave the room for this part of the history. A child’s inability to give a history may be an important observation in itself. Having gained the child’s acceptance, you can now inquire about the problem or complaint.

Key Point

Every school-aged child should be given the chance to speak to the physician alone, a time when the child sometimes reveals information that he or she would otherwise withhold. A child’s inability to give a history may in itself be an important observation.

After allowing the patient to contribute as much as he or she wishes or can, ask the parents to tell you their main concerns. First, establish whether the problem is static or progressive, a distinction that will influence your differential diagnosis and subsequent inquiries. For example, a child with learning difficulties that are worsening is much more likely to have a neurodegenerative or metabolic disorder than a child whose problems are static or nonprogressive. Similarly, an intracranial lesion, such as a tumor, is much more likely if headaches are worsening than if they have been unchanged for months. Always ask details of the child’s school performance because deterioration in grades may suggest a progressive neurological condition.

A brief but detailed history of the pregnancy is essential. Most mothers remember the pregnancy and birth in vivid detail and often have unfounded fears related to it (see Chapter 1). Reassuring a mother that the cough medicine she took during the third trimester was not the cause of her baby’s meningomyelocele may relieve anxiety. Similarly, if a child has been discharged from the neonatal unit at the same time as his/her mother, it is extremely unlikely that significant perinatal problems occurred.

Key Point

Significant perinatal difficulties are extremely unlikely if the child was discharged from the neonatal nursery at the normal time.

The preschool-aged child

Even 2-year-old children like to be involved, and a 3-year-old child may give a more accurate history than his or her distraught parent. Start the history by talking to the patient because many children will soon become bored and distracted. The screening neurological examination of the preschool-aged child is demonstrated in two videos that are associated with this chapter (see Examination Videos 13-1 [girl] and 13-2 [boy] on Student Consult). The young child is most likely to cooperate if you frame requests in a game-like fashion. The preschool child is usually happy to show a disbelieving physician that he or she can count and identify colors. Give the child the opportunity to prove you are wrong. If you announce that Mickey Mouse is pink, even the most sullen child usually corrects you. The child’s success and your delight with his or her performance help improve the child’s confidence in a strange environment.

To have undisturbed time during the subsequent discussions with the parents, it is essential to provide the child with age-appropriate toys. This playtime also provides an excellent opportunity for you to observe the child. Rarely, it may be necessary to have the child removed to another room to play.

Observation

The school-aged child

History-taking offers an excellent opportunity to observe the child unobtrusively. The gait of a child with a hemiparesis may be apparent as he or she walks from the waiting area to the examining room. Typically, the arm is held flexed and adducted against the chest, and the leg is circumducted. The child’s appearance is very important. Note the shape and size of the head (e.g., microcephaly or hydrocephalus), the shape and positioning of the eyes and ears (e.g., in Down syndrome), and any skin lesions (e.g., the facial angioma of Sturge-Weber syndrome). Asking the parents to bring in some family photographs can sometimes be useful. This allows you to compare the child’s appearance with that of his or her relatives.

Key Point

Observe the child’s gait, facial appearance, use of language, and any dysmorphic features during your first encounter.

The formal neurological examination begins during the observation phase. Strabismus or ophthalmoplegia may be apparent. The child’s use of language during interaction with the parents is often more spontaneous and informative than in conversation with the physician. Dysphasia indicates a dysfunction in the dominant hemisphere. Dysarthria may represent problems in the mouth, such as a cleft palate, or may be due to lesions that involve the cranial nerve VII, VIII, IX, X, or XII. Facial asymmetry (as in Bell palsy) may indicate a lesion of the seventh cranial nerve (LMNL) or the cerebral cortex (UMNL).

The preschool-aged child

In the younger child, a period of hands-off observation should always be the first part of the physical examination. I give puzzles, a pencil, and paper to the younger child. Giving children toys allows you to observe the youngster’s manual dexterity, coordination, developmental competence, and handedness (Fig. 13–3). Observe the parent-child interaction closely. You may detect discipline problems at this stage. Drawings also reveal visual-spatial abilities, fine motor skills, and the child’s level of attention. Allowing the child to play also provides important undisturbed time with the parents as you take the history.

FIGURE 13–3 Providing toys for children allows you to observe the child’s hand-eye coordination unobtrusively and relax the child while obtaining a history from the parent.

The infant

The period of observation of an infant is often the most revealing part of the examination. Dysmorphic features may suggest a specific syndrome. As the infant lies in his or her parent’s arms, observe the eye movements, facial movements, and symmetry. The movements of the child might indicate weakness, as is seen in anterior horn cell (AHC) disease (Werdnig-Hoffmann disease). When tone is reduced, the infant may lie with hips and arms abducted (frog-leg position). Infants may appear to be hypotonic (floppy) from either weakness (LMNL) or central causes (UMNL). With floppiness as a result of an LMNL, the infant also has significant weakness and moves very little. The infant who is floppy because of a UMNL is able to move the limbs and is not weak.

Key Point

The hypotonic infant with normal limb movement most likely has central hypotonia (UMNL). The hypotonic infant with weak limb movement most likely has a LMNL, such as a myopathy or AHC disease.

The Physical Examination

For the physical examination, the child’s cooperation is necessary and is usually easy to obtain. If a child with a behavioral problem refuses to cooperate, ask the parents to leave the room; this step is almost never necessary.

The school-aged child

I begin the physical examination of a school-aged child by playing ball with the child, thus extending the period of observation and allowing further evaluation of gait, power, and coordination as the child catches, throws, and kicks the ball.

To test proximal strength, ask the child to hop on each foot. You can also test pelvic girdle strength by asking the child to

1. Arise from the supine position without using the arms

2. Do deep knee bends

3. Duck walk (with hips and knees flexed)

Children with mild spasticity also have difficulty performing these tasks because of their increased muscle tone.

Key Point

Test proximal strength by having the child arise from the floor without using the arms, by doing deep knee bends, and demonstrating the “duck walk.”

Next, have the child walk on the heels and then on the toes. This request is more likely to be successful if you demonstrate by walking on your heels and toes at the same time. Heel walking is an excellent test because weakness of the tibialis anterior (dorsiflexion of the foot) is an early sign of distal weakness in peripheral neuropathies. The patient with marked weakness has footdrop, which may be diagnosed from the slapping sound of the foot as the patient first walks into your office. Normally, the heel strikes the floor first, then the toes.

Key Point

Difficulty walking on the heels suggests distal weakness caused by a neuropathy.

Milder degrees of weakness may require additional testing. To demonstrate mild proximal weakness or spasticity, you may have to ask the child to run up and down stairs. You can easily confirm mild distal problems, however, by having the child repetitively tap each foot quickly on the ground.

Next, test cerebellar function by having the child walk a straight line in a heel-to-toe fashion (tandem gait). Other useful tests of cerebellar function are the pirouette test, during which the child is expected to perform three pirouettes (360-degree turns) while walking. A variant of this test is to ask the youngster to walk several times around an object (such as a chair). In both situations, the child with cerebellar disease will stumble toward the side of the cerebellar lesion. A wide-based “drunken” gait usually indicates cerebellar disease. Tapping each foot repetitively, previously described as a test for distal weakness, is also an excellent test of coordination.

Key Point

Diseases of the cerebellar hemisphere cause stumbling toward the same side as the lesion.

For the Romberg test, the child is told to keep the feet either side by side or in the tandem position, with one foot directly in front of the other. The child is then asked to extend the arms to the front and to close the eyes. Inability to maintain this position indicates a deficit in position sense, called proprioception. With the eyes closed, the child is deprived of visual input and must depend on proprioception to maintain the standing position. Lesions of the cerebellum result in difficulty standing, whether the eyes are open or closed. A loss of balance that occurs only when the eyes are closed is caused by a lesion in either the peripheral nerves or the posterior spinal columns.

Holding the extended and supinated arms in front of the body also tests for weakness. Children with a mild weakness will be unable to maintain this position, and the arms tend to drift downward, flex at the elbow, and pronate (Fig. 13–4).

FIGURE 13–4 Pronator sign.

Check the spine next, while the child touches his or her toes. Look for bony deformities and midline skin lesions, such as tufts of hair or sacral dimples, which may suggest underlying malformations.

Note that up to this point, you have not laid a hand on the child or used a single strange or intimidating instrument. The entire examination has been a hands-off exercise. It is now time to produce your reflex hammer and ophthalmoscope. You should, however, already have a provisional differential diagnosis in mind, which the examination will merely confirm or refute.

Key Point

At the end of the history, you should have formulated a differential diagnosis, which the examination can then confirm or refute.

If the physical examination reveals unsuspected findings, always review your history and observations to decide whether they should have led to different conclusions.

The preschool-aged child

A period of ball playing is also useful for observing preschoolers. Even a child who is reluctant to leave his or her parent’s side will usually play ball. The period of ball playing also helps establish you as an ally. You can then proceed as described for the school-aged child. Remember, however, that 4-year-olds may not successfully complete the tandem gait and that an inability to hop on one foot is not necessarily abnormal in children younger than 7 years.

You can most easily examine children younger than 6 years while they sit on a parent’s lap rather than on the examining table.

Key Point

Examine children younger than age 6 years while they are sitting on the parent’s lap.

Cortical Function

School-aged and preschool-aged children

Assess each cortical area in turn.

Frontal Lobes

The posterior frontal lobes contain the motor cortex. Deficits in a frontal lobe result in signs of upper motor neuron dysfunction on the other side of the body. Motor aphasia appears in children older than 6 or 7 years if the Broca motor speech area in the dominant hemisphere is injured. The frontal lobes also control contralateral eye movements. An irritative lesion in the frontal eye fields (e.g., a seizure) causes a deviation of the eyes away from the side of the lesion; in contrast, a destructive lesion causes a deviation of the eyes toward the side of the lesion.

Disturbance of the more anterior parts of the frontal lobe may cause personality changes, irritability, and lethargy, with a lack of spontaneity. Sphincter incontinence may develop, and primitive reflexes, such as rooting, sucking, and grasp reflexes, may re-emerge. Remember that frontal lobe lesions may be difficult to detect even if they are large.

Temporal Lobes

Temporal lobe impairment may cause personality changes similar to those seen with frontal lobe damage. Language is also represented in this lobe, and lesions of the superior and middle gyri cause Wernicke aphasia, characterized by an impaired comprehension of word elements. The ability to read, write, and understand speech may be altered. If the nondominant temporal lobe is involved, the child has a distorted perception of spatial relationships and a change in musical appreciation. Test for alterations in spatial perception by asking the child to copy geometric designs. Age-appropriate designs are shown in Figure 13–5. Bilateral involvement of the hippocampus interferes with learning. Temporal lobe injury also may produce psychotic aggressive behavior. Visual symptoms are usually represented by a homonymous superior quadrantanopia.

FIGURE 13–5 Shapes used for testing spatial perception.

Memory deficits may be seen with a temporal lobe dysfunction. You can test a child’s immediate recall by reciting number sequences and having the child repeat them after you, either in the same order or in reverse; Table 13–2 gives examples of age-appropriate number sequences for this test.

TABLE 13–2 Test for Immediate Recall*

Key Point

Test a child’s distant memory by asking the names of previous schoolteachers and locations of family vacations over the past few years; test more recent memory by asking what the child ate for supper or what television shows he or she watched the previous evening.

Parietal Lobes

Parietal lobe dysfunction produces sensory perception abnormalities. Two-point discrimination, graphesthesia, and the appreciation of size, shape, and texture are all impaired. You can easily test these perceptions by asking the youngster to identify coins, a tissue, and a paper clip as you place them one at a time in one of the child’s hands while the child’s eyes are closed.

Children with parietal lobe deficits cannot appreciate simultaneous cutaneous stimulation on bilateral homologous body parts. Test this point by asking the child to identify (with the eyes closed) which arm you have touched; the child should be able to identify the simultaneous touch of both arms.

A parietal lobe injury impairs awareness on the opposite side; cortical sensory changes are best tested during the examination of sensation. Assuming that you are neurologically normal, use your own sensory perception as the normal reference.

Lesions of the parietal cortex may also cause apraxia, the inability to perform a series of tasks; apraxia may be present even though the patient can complete individually each component of the action. In young children with damage to the parietal lobes, growth on the affected side is usually impaired. Similarly, a smaller hand in a child with hemiparetic cerebral palsy indicates involvement of the parietal lobe. Such a child has reduced sensation in that limb and, therefore, has more difficulty with hand function than the child with an exclusively motor dysfunction.

Occipital Lobes

Bilateral involvement causes cortical blindness. Unilateral lesions produce homonymous visual field defects.

Cranial Nerves

When testing the cranial nerves, start with the olfactory nerve (cranial nerve I), and sequentially examine each nerve until you have assessed all 12.

Cranial Nerve I (Olfactory)

The school-aged child

The olfactory nerve is responsible for the sense of smell. Your delight at a diagnosis of anosmia will tend to fade, however, if the child’s sense of smell returns after he or she blows his or her nose. Because children seem to have constant respiratory infections, first examine the nostrils. Then, while the child’s eyes are either closed voluntarily or covered by a parent’s hand, ask the child to identify the scent of common objects, such as chewing gum and chocolate, a task he or she should be able to perform. Never use irritants such as ammonia, a detection of which involves the fifth cranial nerve function.

The preschool-aged child

It is seldom possible to perform an accurate assessment of the first nerve in preschool-aged children. If such assessment is clinically indicated, try to perform the technique described for the school-aged child.

Cranial Nerve II (Optic)

Always separately test each eye (see Chapter 8), and divide the examination into the following four parts.

Visual fields

The School-Aged Child

Ask the school-aged child to point to your wiggling finger or to report how many fingers you are extending in each of the four visual quadrants while the child looks straight into your eyes.

The Preschool-Aged Child

For the younger child, test the visual fields by distracting him or her with a toy in front while bringing a red toy from behind the youngster into the peripheral field of vision (Fig. 13–6). The child with normal vision should respond as soon as the toy reaches a line perpendicular to the outer canthus of the eye.

FIGURE 13–6 Visual field testing.

Visual acuity

The School-Aged Child

Visual acuity can be tested in adults, although some children with learning problems (e.g., dyslexia) should be tested with charts that contain images rather than letters (Fig. 13–7).

FIGURE 13–7 Visual charts for young children.

The Preschool-Aged Child

Use age-appropriate vision charts for the child older than 4 years. Test the younger child by offering small, interesting objects. I usually ask the parent to cover one of the child’s eyes while I offer a tiny cake decoration. When the child realizes that it is special candy, he or she readily picks it up. This is also a nice test of fine motor function. I encourage the patient to pick up the candy with each hand.

For less cooperative children, test for opticokinetic nystagmus (OKN) by pulling a striped cloth from one side to the other before the child’s eyes. OKN consists of a slow deviation phase of the eyes in the direction that the stripes are moving, followed by a rapid return (jerk) in the opposite direction. The presence of OKN indicates that the child has cortical vision. This can be a very useful test in children with hysterical blindness. Although they claim to be blind, OKN is present. If OKN is absent in one direction, suspect a hemianopia.

Key Point

Test for OKN by pulling a striped cloth from one side to the other before the eyes. OKN consists of a slow deviation phase of the eyes in the direction that the stripes are moving, followed by a rapid return (jerk) in the opposite direction.

Fundoscopy

The School-Aged Child

It is difficult to distinguish papilledema from papillitis with the ophthalmoscope, but the latter is characterized by a severe diminution in visual acuity. An easy seven-step approach to learning fundoscopy is described in our article, which is referenced at the end of the chapter.

The Preschool-Aged Child

In the preschooler, it is often reasonable to deviate from your usual order of examination by leaving fundoscopy until the end of the examination. The following technique is invariably successful:

1. Seat the child in front of you, and have the parent stand behind you; explain the procedure to the parent. Reduce the ambient light to allow pupil dilatation.

2. Ask the child to tell you whether the parent sticks his or her tongue out.

3. Not surprisingly, the child tries hard to focus on the parent, and shouts when the parent sticks out his or her tongue. Feigning anger with the parent delights the child.

With this technique, the child usually keeps the eyes still, allowing you to view the fundi. If the child cannot maintain fixation, keep the ophthalmoscope still, and view each fundus as it passes before your view while the eyes are moving. If you approach on the horizontal plane approximately 10 degrees to 15 degrees lateral to midline, you will easily see the optic disc.

The Infant

For infants, two techniques are valuable. Allowing an infant to suck on an object (even your finger) usually induces eye opening, and you can view the fundi without difficulty. Alternatively, have the parent hold the baby so that he or she is looking over one of the parent’s shoulders. You can hold the baby’s head gently but firmly while viewing the fundi (Fig. 13–8).

FIGURE 13–8 Fundus examination in an infant.

Pupillary response

The pupillary response is subserved by two cranial nerves, II (afferent) and III (efferent). Test this response in children from all three age groups by moving a focused flashlight beam onto the pupil from the side; as the light is then moved to the other pupil, look for a change in the pupillary diameter. This swinging flashlight test compares the function of the two optic nerves. Decreased input through a dysfunctional second cranial nerve results in less constriction of both pupils when the light is shone into the affected eye (relative afferent pupil defect or RAPD). Swinging the light into the normal eye then results in a greater stimulus, and the pupils constrict further.

Cranial Nerves III, IV, and VI (Oculomotor, Trochlear, And Abducens)

The superior oblique muscle is innervated by the fourth cranial nerve, the lateral rectus by the sixth cranial nerve, and the other muscles—medial rectus, superior rectus, inferior rectus, and inferior oblique—by the third cranial nerve.

The school-aged child

Having the school-aged child follow a flashlight with his or her eyes allows you to see the light reflection, which should be centered on both pupils. If the patient has diplopia, ask him or her to report where the two images are most widely separated. Children may find it easiest to represent their diplopia by holding a pen in each hand, which they can use to demonstrate the position of each image. By having the child close each eye sequentially, establish which eye is seeing the outer (false) image. This maneuver establishes which muscle is paretic. For example, if the images are most widely separated when the child is looking horizontally to the left, and the outer image disappears when the left eye is closed, the left lateral rectus muscle is responsible—signifying a left sixth nerve palsy.

Key Point

Because the sixth cranial nerve has a long course through the cranium, it is especially vulnerable to traction or pressure. Sixth nerve palsy does not help localize the site of dysfunction; it may occur with increased intracranial pressure (ICP) of any cause.

The preschool-aged child

For children younger than 6 years, I place a pencil flashlight inside a plastic finger puppet. Every child readily focuses on this toy, which I move through lateral, medial, upper, and lower visual fields to observe the eye movements.

The infant

Babies’ eyes should follow an object, especially a face, that is moved from side to side at a distance of about 18 inches. In the neonate, eye movements can be tested with the doll’s eye reflex, in which movement of the head results in a deviation of the eyes in the opposite direction. This reflex remains present in a coma and when fixation is prevented.

Ptosis may reflect a deficit of the third cranial nerve but also occurs in neuromuscular diseases such as myasthenia gravis. When ptosis is due to a third nerve palsy, the pupil on the affected side may be enlarged.

Key Point

Remember that in double vision, the outer image is always the false one, and the images are most widely separated in the direction of action of the involved nerve and muscle.

Cranial Nerve V (Trigeminal)

The trigeminal nerves are responsible for the muscles of mastication and for sensation to the face.

The school-aged child

Check the masseter muscles by asking the school-aged child to clench his or her teeth together as hard as possible. The strength of these muscles is impressive, showing why it is senseless and dangerous to try to pry open the mouth of a patient who is having a seizure. Sensation can be tested when other sensory modalities are being assessed. Test all three divisions: ophthalmic, maxillary, and mandibular.

Test the jaw jerk reflex by placing your thumb over the child’s chin (his or her mouth should be slightly open) and then tapping on your thumb; the reflex stretches the masseter muscles, resulting in jaw closure. Both the afferent and efferent pathways are through the fifth cranial nerve. In children, the jaw closure is normally difficult to see, and a brisk closure of the mouth suggests a UMNL. Do not routinely elicit this reflex; even the most trusting child will seriously question your motivation if you approach his or her face with a hammer.

Peripheral lesions of the fifth nerve are rare. Central involvement (UMNL) may occur in the cavernous sinus, where the third, fourth, and sixth cranial nerves may also be involved. Tumors at the cerebellopontine angle may involve the fifth, seventh, and eighth nerves and the cerebellar outflow tracts. Unilateral fifth nerve lesions result in a deviation of the jaw to the paretic side.

The preschool-aged child

In younger children, palpate the masseter muscles while the child chews.

The infant

The masseter muscles can be palpated in infants as they suck.

Cranial Nerve VII (Facial)

School-aged and preschool-aged children

The facial nerve is responsible for the muscles of facial expression. Observing the face during the examination usually allows an excellent assessment of this nerve. Ask the child to forcibly close his or her eyes, wrinkle his or her forehead, and show his or her teeth. The facial asymmetry and weakness are not apparent during emotion-related movements, such as smiling and crying. A UMNL of the seventh nerve (above the level of pons) spares the forehead muscles. Most young children can be persuaded to imitate forced eye closure and to blow on a finger puppet or a flashlight. Children with a facial palsy tend to drool from the weak side of the mouth.

All three age groups

The corneal reflex depends on the fifth (afferent) and seventh (efferent) cranial nerves. Test this reflex by touching the cornea with a wisp of cotton, which is brought to the eye from the side, outside the field of vision. A substitute for this unpleasant test involves blowing gently over the cornea of one eye while your hand protects the other eye from stimulation (Fig. 13–9).

FIGURE 13–9 Blow gently over the cornea while your hand protects the other eye.

Testing taste from the anterior two-thirds of the tongue (supplied by the chorda tympani) is not part of the routine neurological examination in children.

Cranial Nerve VIII (Cochlear and Vestibular)

The school-aged child

Test the acoustic nerve in a school-aged child by observing the response to rustling paper or finger rubbing. I usually test hearing by whispering numbers at a distance of 6 to 8 feet while the child or a parent occludes one of the child’s ears.

You can perform the Rinne test in children older than 6 years by holding a vibrating tuning fork (256 Hz) against the mastoid process (testing bone conduction). When the child can no longer hear the sound, hold the tuning fork close to the external auditory meatus (testing air conduction). Normally, air conduction is perceived for twice as long as bone conduction.

The Weber lateralizing test can also be performed in an older child. Apply the vibrating tuning fork to the midline of the forehead, and ask the child whether he or she hears a sound in the center of the head or in one ear only. Normally, the sound is perceived in the midline. In ear disease, however, the sound seems louder on the affected side because the normal environmental sounds decrease the apparent intensity of the tuning fork on the normal side. In neural deafness, the sound seems to be coming from the normal ear. If the history suggests a hearing deficit, arrange for a formal audiologic evaluation.

The preschool-aged child and the infant

If the history of a preschool-aged child or infant suggests a hearing deficit, arrange for audiologic testing. Parents may report that the child prefers the television volume louder than other people do or that the child is surprised when the parent suddenly comes into the field of vision. You can stimulate the vestibular nerve in a young child by holding him or her in your arms and rotating through 360 degrees, both clockwise and counterclockwise. Eye deviation in the direction of rotation and nystagmus in the opposite direction are normally seen. I believe this is a valuable test because it evaluates a relatively wide area of the brainstem connecting the vestibular and the oculomotor nuclei.

Cranial Nerves IX And X (Glossopharyngeal and Vagus)

The school-aged child

Testing of the 9th and 10th cranial nerves is usually limited to the observation of palatal movement. Have the school-aged child say “Ah” with the mouth held widely open. Examine the gag reflex if there is any suggestion of dysfunction in the history or other aspects of the examination. Early in your career, assess the gag reflex in all patients undergoing neurological assessment, but remember to stand to the side because an occasional child with an excessive gag reflex will vomit.

The preschool-aged child and the infant

The younger, uncooperative child inevitably cries at some stage during the examination; take the opportunity to observe the palatal movements. As in the older child, examine the gag reflex if the history or other aspects of the examination suggest dysfunction. Children who recurrently aspirate milk or food may have an underactive reflex, whereas those with cerebral palsy may gag easily because of a hyperactive reflex. Those with a defective palatal movement have difficulty making the b, d, and k sounds.

Nerve XI (Spinal Accessory)

The school-aged child

Assess the accessory nerve, which supplies the sternomastoid muscles, by having the school-aged child push against your hand with his or her mandible. The contracting sternomastoid on the opposite side can be palpated. Children love success, so give lots of encouragement.

The preschool-aged child and the infant

Test cranial nerve XI in the young child or infant by placing him or her in the supine position. While supporting the shoulders but not the head, push the head laterally. The child will resist the movement, and you can palpate the sternomastoid muscle.

Nerve XII (Hypoglossal)

The school-aged child

Assess tongue movements by asking the child to imitate you as you move your tongue from side to side. Fasciculations of the tongue while at rest indicate AHC disease.

The preschool-aged child and the infant

The examination of the 12th cranial nerve in the young patient is limited to observation when the mouth is opened.

Physical Examination of the Trunk and Extremities

The child must be asked to undress for the physical examination. It is more acceptable to undress the upper and lower halves of the body at different times. I usually examine the exposed legs first, and I ask all families to bring shorts to the consultation. The child then dresses the legs again before undressing the arms and trunk. All children, regardless of age, deserve to have their modesty respected.

Lower extremities

Power

If you follow the examination procedure given in the chapter, you already tested power in the lower extremities when you had the patient hop, “duck-walk,” and walk on toes and heels.

Observe the muscle bulk for wasting. In neuropathies, the wasting occurs distally and can produce pes cavus (Fig. 13–10). Diagnose pes cavus when light is seen under the arch, despite pressing a hard object, such as a book, firmly against the sole of the foot. A hypertrophic appearance of the calf muscles in conjunction with proximal muscle weakness suggests muscular dystrophy (actually pseudohypertrophy because of fatty infiltration of muscles). These pseudohypertrophic muscles feel “rubbery” to palpation. Fasciculations as a result of spontaneous contractions of muscle fiber groups are seen primarily with AHC dysfunction but also occasionally with neuropathies. They can be heard as crackling sounds when you listen over the muscle with the bell of your stethoscope.

FIGURE 13–10 Pes cavus.

The School-Aged Child. Undertake more formal testing with the school-aged child lying supine. Test each muscle group separately. It is useful to use a grading scale, but it is essential to document the scale you have used. I frequently find notes in charts that indicate that the muscle strength in a patient’s biceps was “3/6.” What does this mean? One commonly used scale is outlined in Table 13–3. As part of the routine examination, you should test

1. Hip flexors, extensors, adductors, and abductors

2. Knee flexion (hamstrings) and extension (quadriceps)

3. Ankle dorsiflexion, plantar flexion, inversion, and eversion

TABLE 13–3 Scale for Grading Muscle Strength

0	No muscle contraction
1	Flicker of contraction
2	Movement with gravity eliminated
3	Movement against gravity
4	Movement against resistance
5	Normal power

The Preschool-Aged Child. Place young or uncooperative preschool-aged patients on the floor. Even the most stubborn child eventually gets up. As he or she arises, assess the leg strength. Proximal weakness is characterized by the use of the arms to help the child “climb” up the legs (Gower sign) (see Chapter 15). Children older than 3 years should be able to stand briefly on one foot. A child who is reluctant to perform these tasks usually cooperates if you do the tasks at the same time. If this approach does not work, remember that every child rises to tiptoes to reach for an interesting toy. These maneuvers allow the avoidance of the difficult question of what is acceptable strength for a child. All children should be able to support their body weight.

Key Point

All children should be able to support their body weight.

Tone

The School-Aged Child. If you are following the examination procedure given in the chapter, you have also noted muscle tone during your observation of the patient. It is difficult for students to appreciate the variations of acceptable muscle tone. The normal child younger than 12 years can passively touch his or her nose with the large toe without discomfort. In the spastic child, this maneuver is impossible, whereas the floppy (hypotonic) child can put his or her toe behind the ipsilateral ear. Normally, the ankle can be dorsiflexed 20 degrees, and the hips can be abducted 60 degrees or 70 degrees. Test clonus at the ankles by rapidly dorsiflexing the foot once and hold in the dorsiflexed position. This is reliably done only if the ankle is relaxed. Holding the foot while talking to the child allows him or her to relax eventually. More than two or three beats of clonus is usually abnormal.

The Preschool-Aged Child and the Infant. When the young child or infant is lifted in the air, the “scissoring” of the legs (Fig. 13–11) indicates increased tone (spasticity, UMNL) in the hip adductors. Because of tightness in the hip adductors, the child with increased tone also tends to sit on the floor in the W position, with the thighs and knees touching each other.

FIGURE 13–11 Scissoring of the legs.

Key Point

“Scissoring” of the legs is an early sign of spasticity.

Coordination

The School-Aged Child. Test coordination in cooperative school-aged children by having them run each heel along the shin of the other leg and then tap the knee two or three times with the heel. Repetitively tapping each foot on the ground, as noted previously, is also an excellent test of lower extremity coordination.

The Preschool-Aged Child. Most preschoolers will walk around a chair if the exercise is framed in a game format whereby the child must follow his or her parent.

Key Point

Diseases of the cerebellar hemisphere cause stumbling to the same side as the lesion.

Reflexes

School-Aged and Preschool-Aged Children. It is easy to elicit knee reflexes when you sit opposite the school-aged or preschool-aged child and rest his or her feet on your own knees. The angle at the child’s knees should be symmetric and approximately 120 degrees. With the legs in this position, it is easy to demonstrate the reflexes by tapping just below the patella (Fig. 13–12). I discourage the use of tomahawk reflex hammers. Those with a wheel at the top work best.

FIGURE 13–12 Position for testing the knee reflex.

You can easily obtain the ankle reflex by holding the foot at 90 degrees with your thumb on the plantar aspect and your other fingers over the dorsum. The reflex can be seen when you strike your own thumb with the hammer. If you find it difficult to elicit an ankle reflex, ask the child to kneel on the examining table or chair with his or her heels over the edge (Fig. 13–13), and tap over the Achilles tendon. Before concluding that a reflex is absent, try the same maneuver while you have the child clenching his or her teeth as hard as possible and trying to pull his or her interlocked fingers apart. Reflexes with a slow relaxation phase (especially ankle jerks) are seen in children with hypothyroidism.

FIGURE 13–13 Position for testing the ankle reflex.

Key Point

Areflexia is present only if reflexes cannot be elicited with reinforcement.

The Babinski sign was first described by Joseph Babinski in 1896. For this test, the leg should be slightly flexed and relaxed. With a relatively sharp object, carefully stroke the lateral border of the sole of the foot from the heel to the little toe. A positive response, indicative of corticospinal disease (UMNL), consists of dorsiflexion of the great toe and flaring of the other toes. My thumbnail and a car key have served me well for this test. Numerous other maneuvers that elicit the same reflex can occasionally help if the interpretation of the response is hindered by either a grasp response or an intolerance of the procedure. I find that stimulating the dorsum of the great toe with a pin or firmly rubbing the anterior tibial region (Oppenheim reflex) is helpful.

There has been much debate regarding the significance of the Babinski sign in children younger than 1 year. In general, it should be considered significant only if accompanied by other evidence of a UMNL. In infants, test this reflex with the

1. Child lying supine

2. Head maintained in the midline to eliminate the asymmetric tonic neck reflex (ATNR)

3. Knee extended

4. Foot held at a 90-degree angle

If these precautions are taken, the great toe plantar flexes in the vast majority of infants, as it does in older children.

Test the abdominal reflexes, subserving thoracic segments T8 to T12, while the child lies supine on the bed or examining table. Draw a pinwheel from the lateral aspects of the abdominal quadrants toward the umbilicus, a maneuver that normally induces a movement of the umbilicus toward the stimulus. If indicated, examine the cremasteric reflex (L1, L2) by stroking the inside of the thigh with a sharp object and observing for the retraction of the testis on that side. Another reflex that is sometimes valuable is the anal reflex (S3, S4, S5), or anal “wink,” which is tested by scratching the skin around the anus and observing the contraction of the anal ring. The absence or asymmetry of these reflexes indicates corticospinal tract involvement.

Infants. It is important to assess the primitive reflexes in all children younger than 1 year. Although there are many such reflexes, the Moro reflex and ATNR are the most helpful.

• The Moro reflex first appears between 28 and 32 weeks’ gestation and disappears between ages 3 and 5 months. Elicit this reflex by suddenly dropping the baby’s head 6 to 12 inches and catching it in your hand. The arms abduct with the extension of the fingers, followed by adduction of the arms across the chest and flexion of the hips. This reflex is present in all babies during the first few months, but it normally disappears by 6 months. Look for any asymmetry between the limbs, which is also abnormal.

• Obtain the ATNR by turning the baby’s head to one side. The arm and leg become relatively more extended on the side to which the baby looks. The limbs on the occipital side are flexed; this is also termed the fencer position (Fig. 13–14). The ATNR reflex is also seen during the first 6 months of life but is most prominent during the second and third months. It is impossible for the baby to sit if this reflex is still present, and its persistence (while the baby is awake) beyond 6 months is abnormal. Also consider the ATNR abnormal if the child cannot “break” the posture within a minute or if there is a persistent difference between the responses obtained with the head turned to either side.

FIGURE 13–14 Asymmetric tonic neck reflex (ATNR).

The palmar grasp reflex is present at about 28 weeks’ gestation and is replaced by the voluntary grasp between ages 2 and 3 months. With frontal lobe dysfunction, this reflex may reappear.

Sensation

The School-Aged Child. Although the sensory examination is often considered the most difficult part of the pediatric neurological examination, an easy and accurate assessment is possible in most school-aged children. Using a pin, needle, Wartenberg wheel, or broken tongue depressor for testing pain sensation in children usually causes fear and may end the examination prematurely. I use a nonthreatening pink tracing wheel, an instrument commonly used for sewing. Ask the child to close his or her eyes and to say “Now” whenever he or she is touched and to report qualitative changes in the sensation in different parts of the limb.

If you suspect a hysterical sensory loss, ask the child to say “Yes” when you touch him or her and “No” when you do not. It amazes me how often this approach is successful. The child says “Yes” for the first few touches and replies “No” for the next few, helping persuade the parents that a more detailed review of the stresses in their child’s life is warranted.

Test vibration sense using a tuning fork (128 Hz) placed over a distal phalanx. Ask the child to tell you when the vibration stops, and compare this response with your own ability to perceive vibration. To test proprioception, secure the toe or finger by holding its lateral aspects and move only the most distal phalanx. A tiny movement of 10 to 20 degrees is normally appreciated (Fig. 13–15).

FIGURE 13–15 Testing proprioception.

The Romberg test also assesses proprioception. Ask the child to stand with his or her feet together. For a more challenging variant, ask the child to place his or her feet in the tandem position with one foot in front of the other in a heel-to-toe position.

The Preschool-Aged Child. Allow the child to play with the tracing wheel during the history-taking, so the tool is readily accepted at this stage of the examination. I distract the young child with a toy while I run the tracing wheel up his or her arm or leg. A child with a normal sensation immediately looks at the stimulation site. If the child seems unaware of the stimulation distally but becomes more aware as the wheel is moved proximally, mark the point of apparent awareness. Repeat the test. If the findings are reproducible, further tests to diagnose a neuropathy, such as an electromyogram and nerve conduction studies, are worthwhile.

Perform sensory testing for touch with a paper tissue, being careful not to move the tissue over the skin because it will result in a tickle, which is conveyed through less pure sensory pathways. Younger children can report only the presence or absence of sensation, whereas the older child can report qualitative changes.

To test vibration, place the nonvibrating tuning fork over the bony prominence of a toe or finger, repeating the procedure until the child becomes accustomed to the sensation and no longer reacts to it. Then place the vibrating tuning fork in the same position; if vibration sensation is intact, the child’s response changes.

Perform the Romberg test in a preschool-aged child by having the parents cover the eyes of the child, who is told to stand with his or her feet together. Proprioception is not tested routinely in the young child at this stage because the Romberg test has already been used for this aspect of the assessment.

Key Point

Respect the child’s modesty, but always examine the skin thoroughly for lesions.

Before allowing the child to dress, inspect the skin thoroughly for lesions associated with neurocutaneous syndromes. In neurofibromatosis, there are multiple café au lait spots. In tuberous sclerosis, facial adenoma sebaceum may be mistaken for acne. Patients with this disorder also have depigmented spots (Fig. 13–16) that may have a leaf shape (ash leaf spots). The Sturge-Weber syndrome is characterized by a facial hemangioma that involves the first division of the trigeminal nerve and is associated with underlying abnormalities in the cerebral cortex on the same side. Children with this syndrome often have seizures and mental retardation in addition to other problems. Finally, check the spine for scoliosis and for cutaneous lesions. Children with a tuft of hair or apparent dermal sinus over the spine may have an underlying spinal cord lesion.

FIGURE 13–16 Depigmented lesions.

Upper extremities

Power

The School-Aged Child. Children with chronic hemiparesis (spasticity involving the arm and leg on the same side) usually have a smaller thumbnail on the affected side. Similarly, the nail on the large toe is smaller in even mildly hemiparetic limbs. Establish that each joint has a full range of movement. Test strength more formally by pushing against the abducted arms. Test the biceps with the child’s arm somewhat flexed at the elbow and the hand held in supination. Grip the child’s forearm and ask him or her to resist extension at the elbow. Test the triceps by extending the arm against resistance. Next, assess wrist extension and flexion by pushing over the dorsum and palmar aspects of the hands, respectively. Examine finger strength in the older child as in the adult. If you allow the child to “win” some of these contests of strength, he or she is encouraged to make greater effort, allowing you to more accurately assess muscle strength.

Key Point

Chronic hemiparesis is associated with a smaller thumbnail on the affected side.

The Preschool-Aged Child and the Infant. Test upper extremity strength in the younger child by staging a wheelbarrow “race” (Fig. 13–17). In this maneuver, the arms support the total body weight.

FIGURE 13–17 Wheelbarrow position.

You can easily examine shoulder adductors by lifting the young child with your hands under the axillae. Assess distal strength by getting the child to pull a toy from you. In the child younger than 5 years, test the proximal power of his or her arms by holding the child under the axillae without supporting his or her chest. Your hands act as hooks; the child should be able to support his or her own weight.

Tone

The School-Aged Child. Test tone in the upper extremities of the school-aged child by holding his or her hand and alternately pronating and supinating the forearm. Increased tone in the arms is often most apparent in the hands, where the thumbs are held tucked in under the fingers in a fisted position (Fig. 13–18). Your confidence in distinguishing normal and abnormal tone depends on the number of children you have examined. Take every opportunity to assess muscle tone in your pediatric patients, and you will quickly appreciate the range of normal muscle tone in children.

FIGURE 13–18 Fisting.

The Preschool-Aged Child. If the preschool-aged child is uncooperative, you may assess the tone more easily by holding each forearm and then shaking it to observe wrist movements. If you accompany these movements with silly noises, the child is more likely to cooperate. This maneuver is difficult to resist and is a more reliable way to assess muscle tone in the young child.

The Infant. Hold the infant in horizontal suspension. The floppy baby hangs like an inverted U (Fig. 13–19), whereas the spastic child hyperextends his or her trunk. When pulled to sit, the normal neonate shows some evidence of neck flexion. The scarf sign is a helpful test for upper extremity tone; to test for it, adduct the arm as far as possible. In the normal infant, the elbow can be brought to midline, touching the chin.

FIGURE 13–19 Prone floppy baby.

Coordination

The School-Aged Child. Ask the school-aged child to touch your finger and then his or her nose. Be sure to make him or her stretch to reach your finger because tremor may be apparent only during the last few centimeters of extension.

The Preschool-Aged Child. Coordination is assessed easily and accurately in a child who is 18 months or older by asking him or her to press a penlight bulb with his or her index finger or great toe. I present this request as a “magic test.” When the child touches the light bulb, switch off the light. The young child believes he or she has magically turned off the light and is delighted. The more mature child is pleased to let you know that he or she has not been fooled by such a simple trick. This test is as accurate as any performed in the adult.

Test fine motor function by asking the child to screw a nut and bolt together, to pile a series of blocks, and, for the older child, to sequentially approximate the thumb with each finger. Difficulty performing rapid repetitive movements (tapping the hand on the leg) and alternating movements (pronation-supination, as in screwing in a light bulb) may indicate cerebellar disease or dyspraxia. Dyspraxia, the inability to perform a series of tasks despite being able to do each segment individually, is usually the result of parietal lobe disease.

Reflexes

School-Aged and Preschool-Aged Children. Examine the deep tendon reflexes in a school-aged or preschool-aged child as you would in an adult. For the biceps reflex, the child’s arms should rest in his or her lap with the elbows partially flexed. I place my finger over the tendon and then hit my own finger, thus persuading the child I do not want to hurt him or her and also allowing me to feel muscle movement during the reflex. You can more easily test the triceps reflex by abducting the arm 90 degrees with the elbow flexed to 90 degrees (perpendicular to the ground) (Fig. 13–20). In this position, the reflex can be seen easily, and it is difficult for the child to resist. Elicit the Hoffman reflex by holding the patient’s hand in the pronated position and flicking down the terminal phalanx of the middle finger. In children with UMNL, the thumb flexes and adducts, and the other fingers also flex.

FIGURE 13–20 Position for testing the triceps reflex.

Sensation

Testing sensation in the arms is as described previously for the legs.

Then, auscultate the head for bruits. As many as 60% of normal children 4 to 5 years old and 10% of children 10 years old have audible intracranial bruits that are of no clinical significance. Benign bruits are softer and less harsh than bruits associated with vascular lesions. The only way to confidently make this distinction is to listen to the cranium of every child you examine. Measure the head circumference and palpate the anterior fontanelle and cranial sutures.

Now that you have read over the entire procedure for neurological assessment, try applying this approach to the following case histories.

Case Histories

Case History 1

History. Six-year-old Peter is brought to see you because he frequently trips when running.

What questions will help you form a reasonable differential diagnosis?

First, you must establish whether Peter’s problems are acute or chronic. When the parents are asked about his developmental milestones, they say that he first walked at 22 months and that he has always been clumsy. He has difficulty riding a bicycle, and he becomes frustrated when he cannot keep up with his friends. The family does not think his problem is getting worse, but it is certainly not getting better. He has not lost any skills.

At this stage, you have evidence that Peter does not have a neurodegenerative condition, but you do not know whether his problem is due to a UMNL or LMNL. Further questions about his development may help sort out whether his problems are related to weakness (LMNL) or central deficits (UMNL). His parents say that he had no difficulty speaking, but he did not crawl until he was 11 months old. His social skills have always been normal. The history, therefore, suggests a normal cerebral cortex but does not exclude damage to a restricted part of the cortex.

Because alterations in tone help distinguish central (UMNL) from peripheral (LMNL) deficits, you ask the parents whether they have ever noticed that Peter’s legs were different. They report that they have always believed his right leg was stiffer than his left leg. This suggests spasticity—a UMNL—despite the otherwise normal language and social development.

The presence of spasticity that is not getting worse indicates a diagnosis of cerebral palsy (CP), which is defined as a nonprogressive disorder of movement with onset in early childhood.

Were there any problems at birth to suggest a cause?

Although perinatal difficulties occasionally can give rise to cortical damage and subsequent CP, it is now clear that, at most, only 8% to 14% of cases of CP are caused by perinatal problems. Most are due to prenatal problems (before birth). Chorionitis is a possible prenatal precursor of CP and is much more common in premature babies.

Key Point

Cerebral palsy is seldom caused by perinatal problems.

Like Peter, most children with CP are the products of normal pregnancy, labor, and delivery. Peter’s parents report he has always been healthy. They say he is clumsy but not weak, further evidence that he does not have an LMNL problem.

What is your differential diagnosis?

Although a delay in achieving motor milestones is most often caused by a global developmental delay, Peter’s normal speech and social skills exclude this possibility. The history does not suggest an LMNL as a cause of his tripping, and the parents report that he has not lost any skills, thus ruling out neurodegenerative diseases. One should still consider the possibility of hydrocephalus, but the differential diagnosis should focus on possible causes of CP. These can be divided into prenatal, perinatal, and postnatal problems. Prenatal difficulties may be suggested by signs of congenital malformations or anomalies. CP involving one side of the body (hemiparesis) may be the result of a fetal vascular insult (stroke) during pregnancy. Perinatal problems are usually excluded if the patient was discharged from the neonatal nursery at the usual time. Children who suffer an insult in the perinatal period sufficient to result in long-term neurological impairment always have neurological difficulties as neonates. Postnatal causes of CP include trauma and infection, such as meningitis. These are easily excluded by means of a thorough history.

What should you observe?

As you watch Peter, you notice that he tends to turn his right foot in and swing his leg from the hip (circumduction) when he runs ahead of you in the hall (Fig. 13–21; Video 13-4 on Student Consult). While drawing, he holds the pencil in his left hand and keeps his right arm flexed tightly across his chest, supporting your suspicion that he has a hemiparesis. He has no evidence of weakness as he rises from the floor. Your conversation with him supports his mother’s claims that he is a smart little boy.

FIGURE 13–21 Hemiparetic gait.

Examination of the wear patterns on the bottom of the shoes shows that the right shoe has signs of the unusual wear of the right toe, caused by scraping the foot along the ground.

What aspects of the physical examination deserve particular attention?

The cranial nerve examination shows a right homonymous hemianopia. This is an important finding because Peter’s inability to see objects on his right side will influence practical aspects of his life, such as where he should be seated in school. The rest of his cranial nerves are normal.

Peter’s muscle power is normal, but the tone is increased in his right arm and leg, and he has hyperreflexia on the right. His right plantar response is upgoing. Sensation in his right hand appears to be decreased, and the nail beds of the thumb and great toe are smaller on the right than on the left. This difference in nail size suggests the chronic nature of Peter’s problems and is associated with the involvement of the parietal lobe.

Peter’s coordination is hindered by the changes in tone, but there are no findings to suggest cerebellar disease.

What is your differential diagnosis, and how will you manage this patient?

All of the findings point to a lesion in the left hemisphere and, with the history of early onset and no progression, your diagnosis of CP is most likely correct. A magnetic resonance imaging (MRI) scan is worthwhile because it may indicate the origin of Peter’s CP. On clinical grounds, you predict that the left hemisphere will be found to be smaller than the right and that there may be a porencephalic cyst in the left hemisphere. The latter is a result of the prenatal insult that damaged the left hemisphere. The MRI confirms your clinical suspicions but does not add to your knowledge of the pathogenesis of Peter’s CP. You counsel Peter’s parents that his problems did not begin at the time of birth and that the parents themselves were not responsible for his CP. This is a topic that you will probably have to address again with them in the future.

Case History 2

(See associated Video 13-4 on Student Consult.)

History. George is a 10-year-old boy who has recently moved to your area. His parents are concerned that he has problems keeping up with his friends in physical activities.

What are your first questions to establish a differential diagnosis for George?

As in Case 1, you should initially ascertain whether George has a progressive or static condition and whether he has a UMNL or an LMNL.

The family thinks George has more difficulty now than a year ago, suggesting a progressive process. They also think he is weaker than the other children, indicating a probable LMNL. They believe George has most difficulty running up stairs. This may reflect proximal weakness. He has no difficulty unscrewing bottles, suggesting normal distal strength. Remember that neuropathies result in distal weakness. The family does not think George fatigues very easily, although he does not have the same stamina as his friends. Thus, he is unlikely to have myasthenia gravis, a disorder of neuromuscular transmission that is characterized by fatigue. The poor stamina is a nonspecific reflection of weakness.

The remainder of George’s history is unremarkable, but his mother reports that she had a brother who died at age 18 years after many years in a wheelchair.

What other questions would you ask this family? What is your differential diagnosis?

Note that, as in most conditions, the diagnostic clues are in the history. George has progressive weakness, which is probably most marked in his proximal muscles. This suggests a progressive myopathy or disease of the AHCs. He is less likely to have a neuropathy because his distal strength seems relatively intact, and he is unlikely to have myasthenia gravis. Childhood myopathies can most easily be divided into the muscular dystrophies, congenital myopathies, and metabolic and inflammatory myopathies.

What do you expect to see as you observe George?

(See associated Video 13-3 on Student Consult.)

As you walk to the examining room with this family, you notice that George has an unusual gait. His hips seem to swing from side to side (Trendelenburg gait). During the interview, he settles on the floor to play with some toys. When he moves to another area of the office, he has difficulty rising to his feet. He seems to push himself up using his hands on his legs. This, of course, represents the classical Gower maneuver, typically seen in children with proximal muscle weakness.

Despite this apparent weakness, George looks muscular and has particularly big calf muscles. What is the significance of this observation?

What will your physical examination find?

George’s cranial nerves are normal. In particular, there is no evidence of tongue fasciculations. George cannot arise from the floor without arm assistance, and he is unable to “duck-walk” or hop on one foot. He tends to walk on his toes but has a reasonably normal distal strength. His muscle bulk is increased in the deltoid and gastrocnemius muscles. Tone is reduced in the extremities, but he has passive dorsiflexion of the ankles to 90 degrees only. His reflexes are difficult to elicit, especially at the knees, but plantar responses are downgoing. His coordination and sensation are normal. Auscultation of his muscles is normal.

What is your diagnosis at this stage, and how will you proceed?

The examination has confirmed your suspicion of proximal weakness. Your differential diagnosis before the examination was of either AHC or muscle disease. The absence of tongue fasciculations, the absence of abnormal muscle sounds on auscultation, and the presence of deep tendon reflexes all make AHC disease unlikely. George, therefore, is most likely to have muscle disease. The increased tone at his ankles is common in children with muscle disease. When muscles are held in a contracted position for a prolonged period, they eventually shorten, with resultant contractures. George’s muscle hypertrophy in the presence of progressive weakness and the history of a maternal uncle with weakness strongly suggest a diagnosis of Duchenne muscular dystrophy. The diagnosis must be confirmed with genetic studies, muscle biopsy, or both. Although Cases 1 and 2 represented patients with difficulty keeping up with peers, the history and examination make the distinction easy between CP (UMNL) and myopathy (LMNL).

Case History 3

History. Jason, a 6-year-old boy, comes to you because of headaches.

What questions might you ask him?

Remember that the history that you obtain from Jason will be more valuable than that provided by his parents. Table 13–4 provides some guidelines for questions that might be valuable in this situation.

TABLE 13–4 Characteristics of Headache Pain and Possible Meaning

Question	What the Answer May Indicate
When did headaches begin?	Chronic headaches are less likely to be due to significant disease.
Are they getting worse?	Headaches due to increased intracranial pressure often become progressively more severe.
Where are they located?	Migraine headaches switch from side to side.
	Tension headaches tend to occur like a band around the head.
	Headaches due to increased intracranial pressure may always be located in the same position.
What type is the pain?	Migraine is usually described as throbbing.
What type is the pain?	Tension headaches are typically “pressing,” but tumors (increased intracranial pressure) can produce throbbing, pressing, or sharp pain.
When does the pain occur?	Migraine is episodic and often occurs in the afternoon but will occasionally wake the patient.
	Tension headaches may last all day but do not interfere with sleep.
	Pain due to increased intracranial pressure is worse in early morning and may awaken the child.
Does the headache interfere with play?	Migraine and increased intracranial pressure disrupt the child’s activities.
Does the headache interfere with play?	Tension headaches are complained of but do not interrupt anything; the child may use them to avoid unpleasant tasks.
Are there associated symptoms?	Tension headaches are seldom associated with other symptoms.
	Migraine is usually seen with anorexia, nausea, vomiting, photophobia, phonophobia, and visual symptoms such as flashing lights.
	Increased intracranial pressure results in vomiting and may cause diplopia, due to a VI nerve palsy.
What relieves headache?	Migraine is relieved by a brief period of sleep.
	Increased intracranial pressure is seldom relieved by any specific factor.
	Tension headaches are helped by stress reduction.
Is there a family history of headaches?	Such a history is common in migraine and stress headaches.
Has the child shown any changes in personality, ability, or thinking?	Such changes are more likely to be associated with significant disease.
Are the headaches triggered by any foods, activities, or events?	Migraine is often triggered by stress, specific foods, or tiredness.

Jason reports that the headaches began 2 weeks ago and are becoming steadily more severe. In reply to your questions, he says that the pain is both sharp and throbbing, and he locates it by pointing with one finger over his right temporal region. When asked if the pain comes on at any particular time of the day, he confirms that the headache awakens him at about 6:00 a.m.

The information shown in Table 13–4 should make it apparent that these findings are worrisome, suggesting raised intracranial pressure (ICP). The early-morning predominance reflects an aggravation of the slight increase in ICP that normally occurs at night.

Jason vomits with the headaches but he says he does not feel sick, a finding also suggesting raised ICP. Children who vomit with a migraine typically have associated nausea.

Over the past 2 weeks, Jason has not gone out to play with his friends, but he has missed only 2 days of school. In response to questioning, he says he has not been hit on the head.

Do the headaches interfere with play?

Insignificant headaches may prevent school attendance but not participation in sports and other pleasurable activities. Learning more details of a patient’s school performance is often an easy way to document cognitive function. Jason is described by his teachers as an excellent student who is always at the top of his class. He has had difficulty concentrating over the past 2 weeks, probably reflecting the severity of the headache pain. Children who have difficulties in school may have other problems, such as attention deficit hyperactivity disorder or learning disabilities, which require a thorough developmental assessment (see Chapter 6).

Jason tells you that he has sometimes seen double over the past couple of weeks. The remainder of his history is unremarkable.

What is your differential diagnosis?

The history indicates that Jason almost certainly has some lesion that is causing increased ICP. With the information provided, you cannot predict where such a lesion is located, but your differential diagnosis is focused on either a tumor or, possibly, hydrocephalus. Other space-occupying lesions, such as a subdural hemorrhage and abscess, are unlikely because both of these conditions would be expected to cause more profound illness.

What will be of particular interest during the period of observation?

As you watch Jason walk into your office, he appears to have a mild limp, suggesting weakness of his left leg. The family reports that this is a recent change in his gait. His ability to answer your questions and his conversation with you suggest that he has normal intelligence. Similarly, his speech is clear, indicating that he does not have dysfunction of the 9th, 10th, or 12th cranial nerves. Intermittently throughout the history, Jason closes one eye, which he says is due to seeing double (diplopia). This suggests a problem with cranial nerves III, IV, or VI. He seems concerned about his headaches. By contrast, many children with stress or tension headaches report terrible headaches but appear unconcerned as they play in your office.

Has the period of observation helped you localize Jason’s problem?

The presence of a recent change in gait, with left leg difficulties, suggests a problem in the right hemisphere. The diplopia is most likely secondary to a sixth nerve lesion. As noted previously, this is not a helpful localizing sign because the sixth cranial nerve has a long course and is especially vulnerable to traction or pressure.

How will your physical examination help confirm your suspicion of a right hemisphere lesion?

Jason has difficulty running because his left leg tends to turn in. As he runs, you notice that his left arm is held adducted and swings less than his right. He is unable to hop on his left leg, cannot repetitively tap his left foot, and has difficulty with fine movements involving the left hand. He throws a ball better with his right hand, even though he is left-handed. These findings suggest spasticity of the left arm and leg.

Examination of the cranial nerves reveals bilateral papilledema, which reflects increased ICP. His double vision is most marked when Jason looks to the left, and the outer image disappears when he closes his left eye, therefore indicating that the responsible nerve is the left sixth cranial nerve. His power is probably normal, although you are uncertain whether he is slightly weak on the left. His tone, however, is definitely increased in both the left arm and leg, and he has five beats of clonus at the left ankle. The reflexes are also brisker in the left limbs, and the plantar response shows dorsiflexion of the great toe. His coordination is reduced on the left, but you think that this finding is due to the spasticity (increased tone) rather than to cerebellar dysfunction. His sensation is normal.

What is your differential diagnosis now?

All of your findings confirm your initial suspicions and point to an expanding lesion of some sort that affects the right motor cortex. The findings are not specific for any particular disease or lesion.

How will you manage this patient?

Jason requires an urgent computed tomography (CT) or MRI of his head. Scanning demonstrates a tumor in the right posterior frontal lobe. He is referred for neurosurgical consultation and undergoes successful surgery.

Summary

The neurological examination can be made fun for children while allowing a thoughtful analytic approach to diagnosis. The use of simple instruments and toys (Fig. 13–22) can increase the child’s enjoyment of the experience. On completing the evaluation, first hypothesize the location of any deficit in the nervous system that might account for the findings; then try to decide what disease processes could produce such a lesion. Base the need for further investigations on the differential diagnosis, always forming a specific question that each investigation should answer. The most important part of the physical examination is the period of hands-off observation.