A mother brings her 4 year old son into your office for headaches occurring for the past month which are getting worse. In the beginning, he would complain of headaches during the daytime but these would resolve after several hours and he would run out and play. During the past several days, he has been complaining of worsening headache, sometimes waking him from sleep in the early morning, occurring almost every day. These recent headaches have been associated with vomiting and he has been clumsier on the playground. There has been no history of trauma, fever, respiratory symptoms, or visual problems.

Past medical history is unremarkable. His growth and development have been normal.

Family history is significant for migraines in his mother and other relatives.

Exam: VS T 37.0, P 80, R 20, BP 120/80, Weight 17 kg, Height 105 cm, HC 50 cm (all in the 50th percentile). He is a healthy appearing, alert boy in no distress. No unusual odors are present. No rashes or neurocutaneous stigmata are visible. His head is normocephalic and atraumatic. No dysmorphic features are seen. TMs are clear. Throat is normal. Lungs, heart, and abdomen are all normal. There is no sacral dimple on his back.

Neurologic exam: Pupils are equal and reactive to light. Extraocular movement is impaired on lateral gazing of the left eye. The rest of his EOMs are intact. Horizontal nystagmus is exaggerated towards the left, no vertical or rotatory nystagmus is present. Funduscopic examination reveals papilledema without retinal hemorrhages. Visual acuity is 20/30 for both eyes. No facial asymmetry noted when smiling or closing his eyes. Hearing testing with tuning fork is equal on both sides. His gag reflex is intact. He is able to shrug his shoulders and turn his head in both directions. His tongue is midline. His biceps, triceps, deltoids, knee flexors and extensors strength are 5+/5. He is not able to do finger to nose testing. Dysdiadochokinesia is present. He has a stumbling gait. His temperature sensation is intact. He is able to discriminate coins, paper clips, and rubber bands. His deep tendon reflexes are +2/4. No clonus present. He has a negative Babinski.

The history is significant for signs of increased intracranial pressure with headache and vomiting. You wonder about cerebellar dysfunction because of his clumsiness. The physical examination confirms this with papilledema and cerebellar signs with dysdiadochokinesia. You determine that the lesion probably originates from the cerebellum and may be on the left side because of the left eye paresis on lateral gaze and exaggerated horizontal nystagmus to the left. Putting all of this information together, especially remembering that four year olds normally do not complain of headaches and the additional fact that the headaches are severe enough to wake him at night, makes you suspect that this child has a tumor in his cerebellum. An MRI of his brain demonstrates acute hydrocephalus and a cerebellar astrocytoma.

Neurologists are often thought of as detectives since it is their goal to determine where any lesion or problem is in the nervous system. They have to be systematic in their approach in order not to miss anything. By careful history taking and physical examination, they try to determine where the problem is, and only then do they obtain further diagnostic studies to confirm or localize the area of involvement. The pediatric neurologic examination is often challenging to those who have primarily worked with adults since infants and younger children are often uncooperative. This chapter will focus on two major areas of the examination, the history and physical examination.

History. A careful and accurate neurologic history is the most important part of the assessment. In evaluating the history of present illness, it is important to note the onset of symptoms in chronological order, and their frequency, duration, and associated characteristics (1). Also, it would be useful to know whether the problem is static, progressing, or improving. Children who are older than 3 to 5 years may be able to give answers to questions if done appropriately, which may not only be helpful but could be more accurate than the parent's. The review of symptoms is also important since vomiting, fever, clumsiness, and other symptoms can be associated with the presenting problem. A birth history should be obtained, focusing on prenatal, perinatal, and postnatal events. One should ask about the gestational age, complications during pregnancy (including infections), maternal drug and alcohol use, Apgar scores, problems during delivery (such as meconium in amniotic fluid), and feeding difficulties. The past medical history should include immunization status, accidents, chronic medical problems, and medications (including anticonvulsants). A good developmental screening assessment with milestones can be performed (such as the Denver II). Family history can also be useful since some diseases are transmitted through dominant genes such as some neurocutaneous syndromes and migraines, or through recessive genes such as in the case of many neurodegenerative disorders (2).

General Physical Examination. The following is a list of items that should be performed in the general examination (2).

1. Height, weight, blood pressure, and head circumference. If the head circumference is small, then there might be microcephaly. A nice rule of thumb for head circumference is the 3 & 9 rule. A newborn has a head circumference of 35 cm, a 3 month has a circumference of 40 cm, a 9 month has a circumference of 45 cm, a 3 year old has a circumference of 50 cm, and a 9 year old has a circumference of 55 cm (3).

2. General appearance, including dysmorphology.

3. Skin examination for neurocutaneous lesions, such as ash leaf spots, cafe au lait spots, angiomas, axillary freckling, adenoma sebaceum, or shagreen patches.

4. Location of the hair whorl and appearance of palmar creases. Abnormal hair whorl location can signify the presence of cerebral malformations.

5. Quality of scalp hair, eyebrows, and nails. Friable, kinky hair may signify Menkes kinky hair disease that is associated with mental retardation and optic atrophy.

6. Examination of the midline of the back and neck for a sacral dimple, a tuft of hair, or other signs of spinal dysraphism.

7. Comparison of thumbnail sizes and their convexity. Abnormalities may signify a growth disturbance, which may be a sign of hemiparesis.

8. Presence of unusual body odor, which is present in some inborn errors of metabolism.

Neurologic Examination of the Child. The process is the same as that of the adult, although one must remember that children are often frightened of those with white coats and their attention span is rather short. Therefore, the following general tips should be kept in mind:

1. Use items such as a tennis ball, small toys (including a toy car), bell, and an object that will attract the child's attention (like a pinwheel).

2. Do not wear a white coat.

3. Postpone uncomfortable tasks until the end, such as funduscopy, corneal and gag reflexes, and sensory testing.

4. Make the most of every opportunity to examine the child. See how he or she plays, taking into account handedness and motor deficits.

5. Examine the younger child in the parent's lap. Be patient and wait for the child the make the first move before touching him or her. Give the child a toy to establish rapport.

The examination can be summarized in the following steps: 1) Examination of the skull. 2) Cranial nerves. 3) Strength. 4) Cerebellar function. 5) Sensory. 6) Reflexes.

The examination of the skull can lead to the discovery of microcephaly, macrocephaly, and craniosynostosis (or premature closure of the cranial sutures). Prominence of scalp veins may signify increased intracranial pressure. Flattening of the occiput is seen in children who are developmentally delayed, while prominence of the occiput may signify Dandy-Walker syndrome. Ridging of the cranial sutures is a sign of craniosynostosis. Percussion of the skull showing areas of tenderness may signify osteomyelitis. Macewen (cracked pot) sign is where the sutures are separated, which may indicate increased intracranial pressure. Palpation of the anterior fontanelle is also important since one can estimate intracranial pressure. If the anterior fontanelle is bulging, then increased intracranial pressure may be present. The skull can be auscultated using the bell of the stethoscope in six locations for bruits: globes, the temporal fossae, and retroauricular or mastoid areas. Intracranial bruits are heard in many cases of angiomas, which are often accompanied by a palpable thrill. They can also be heard in anemia, thyrotoxicosis, and meningitis (2).

Cranial nerves (2). CN I (olfactory) is rarely tested although it can be by asking the patient to smell something. Olfactory sensation appears at 5 to 7 months of age. CN II (optic) can be tested through various means. Funduscopic examination can be performed with the appearance of the optic disk, macula, and retina noted. An early sign of papilledema is obliteration of the disk margins and absent pulsations of the central veins. Visual acuity can be tested by a vision chart or by offering toys of various sizes to the younger, uncooperative child. Rotating a striped drum or drawing a strip of cloth with black and white squares in front of the eyes can test for optokinetic nystagmus. A homemade drum can be made by attaching a paper with alternating black and white stripes around an empty soda can with a metal wire piercing through it (4). Alternatively, optokinetic nystagmus can be elicited by passing a vertically striped cloth horizontally across the patient's visual field. Optokinetic nystagmus can be elicited starting about 4 to 6 months of age and it confirms cortical vision, in addition to supporting the integrity of the frontal and parietal lobes and visual fields. Visual fields can be tested in children less than a year of age by having one examiner attracting the attention of the child to a toy after which another examiner in back of the child brings another toy into the field of vision, with the location at which the child turns his or her head towards this second toy noted. The blink reflex, which is where an object is rapidly brought close to the child's eyes, appears at about 3 to 4 months. It is present in about 50% of babies at 5 months, and 100% of children at 12 months.

CN III, IV, and VI (oculomotor, trochlear, abducens) can be checked by extraocular movements and pupillary size and reaction to light. Pupils may be large and not responsive to light in babies earlier than 30 weeks gestation. A mnemonic to remember the cranial nerves which innervates the extraocular muscles is the formula (LR6SO4). CN 6 innervates the lateral rectus, CN 4 innervates the superior oblique, and CN 3 innervates the other ocular muscles.

The Doll's eyes phenomenon can also be used to assess extraocular movements in a comatose patient with an intact brainstem. In these patients, horizontal eye movements can be elicited when the head is suddenly turned to one side resulting in the eyes moving to the opposite side in a symmetrical fashion. Also, vertical eye movements can be demonstrated by rapidly moving the head up and down, with the eyes moving in the opposite direction of the head, again in a symmetrical fashion. If a patient were brain dead with no brainstem function, then no Doll's eyes movement would be seen. In effect, the eyes would be stationary when the head is turned. In conscious patients, the cerebrum would interfere with the Doll's eyes phenomenon. Although the Doll's eyes phenomenon can determine whether the brainstem is still functional, a better test would be to do cold calorics. In order to do this test, 5 mL of ice water is squirted into the external ear canal in comatose patients or 0.5 mL in alert, awake patients and the action of the eyes are noted. There are three possible responses to this test. In the comatose patient with an intact brainstem, the eyes move in the direction of the stimulus. In alert, awake patients, there is nystagmus with the quick component in the opposite direction of the stimulus. Lastly, in patients without a functioning brainstem, there is no movement of the eyes when cold calorics are performed. One needs to remember that cold calorics do not test for extraocular movements, but for vestibular function (CN VIII).

A special note about pupils is inserted here because of a common documentation concern. PERRLA stands for pupils equal round and reactive to light and accommodation. Some individuals write PEARL which might stand for pupils equal and reactive to light. The latter is probably satisfactory although it might be better to write PERRL (leave out the A). The accommodation reflex is difficult to see on light-eyed individuals, it is impossible to see on dark-eyed individuals, and it is impossible to accomplish on non-cooperative subjects such as infants and toddlers. Thus, to write PERRLA on a 6 month old or a comatose patient is an obvious error. If you are in the habit of writing PERRLA, ask yourself this. Do you really check the accommodation reflex and did you really, truly see the proper reaction? What is the clinical utility of checking the accommodation reflex? It has almost no clinical utility other than to identify the Argyll-Robertson pupil of neurosyphilis. In the medical malpractice literature, a chart entry such as "PERRLA" has been used to discredit the physician's credibility since a jury can be easily convinced that the accommodation reflex was not or could not be done. If you still believe that you must do the accommodation reflex, then you should write "PERRL + intact accommodation" and note the patient's eye color, to show that you really did it. A medical student once saw a patient for fever and in the past medical history; he wrote that the patient has a glass eye because of a traumatic injury. The exam section of his note indicated: PERRLA. Be careful, the pupil of the glass eye does not react to light or to accommodation.

CN V (trigeminal) is assessed by checking the sensation of the face. Noting the action of the temporalis and masseter muscles can test the motor roots of this cranial nerve. The corneal reflex also checks the ophthalmic branch of CN V. CN VII (facial nerve) dysfunction is seen with facial asymmetry. Taste in the anterior two-thirds of the tongue is innervated by the chorda tympani branch of VII, and can be checked by applying salt or sugar solutions by cotton-stick applicators. CN VIII (auditory) which conducts cochlear and vestibular function can be tested by the child's response to a bell or by recalling a whispered word or number. Noting the eye movements after turning the child several times in a clockwise and counterclockwise direction can check vestibular function. CN IX, X (glossopharyngeal, vagus) function is determined by the position of the uvula and palate. If there is a vagal nerve problem, the uvula will deviate toward the unaffected side, and the palate will move away from the affected side. The gag reflex actually tests parts of IX and X, since IX is the afferent sensory limb (sensory to the back of the pharynx) and X controls the muscles of the pharynx and elevation of the palate. CN XI (spinal accessory) is tested by asking the patient to turn his/her head against resistance, which involves the sternocleidomastoid muscle. CN XII (hypoglossal) dysfunction is seen when the tongue deviates toward the affected side.

The above tests should be considered basic. Several cranial nerves cannot be tested fully since they have multiple functions. For example, the multiple functions of CN X (vagus) cannot be tested fully and we really do not check CN IX's sensory limb from the carotid sinus function. Thus it would be less than truthful if one wrote down "cranial nerves intact,” “CNs II-XII intact,” or “cranial nerves grossly intact.” These statements are actually assessments rather than descriptions of observations which are what the physical exam should state. Additionally, it would not be possible to honestly state that the cranial nerves are intact since several cranial nerve functions were not tested (because they cannot be easily done). For example, to assume that CN X is intact just because the patient gags is a bit of a stretch. The term "grossly intact" usually means that a cranial nerve exam was not done, but the patient's facial function is symmetric. Perhaps it would be more honest to state that the “the patient's facial function is symmetric.” In the medical malpractice literature, statements such as “CNs II-XII intact” have been used to question the credibility of the physician's chart entries since a jury can be easily convinced that an exam to support such a statement is not easily done.

Motor System (2). Observing the child's posture and simple maneuvers such as retrieving a ball or running outside the examination room can check motor integrity. The following grading system can be used for assessing muscle strength:

0 - No muscle contraction

1 - Flicker or trace of contraction

2 - Active movement without gravity

3 - Active movement against gravity

4 - Active movement against gravity and resistance

5 - Normal strength

A sensitive test to assess the strength for the upper extremities is the pronator sign. Have the child raise his/her arms and note the position of the arms. Weakness of the arm is seen by hyperpronation and elbow flexion. Strength of the flexors of the knee can be tested by the Barré sign, which is performed by having the child keep both knees at right angles while lying prone.

Cerebellar function (2). Observing how a child reaches for and manipulates toys can check for coordination. Other tests include finger-to-nose and heel-to-shin. Rapid pronation and supination of the hands or rapid tapping of the foot can assess for dysdiadochokinesia, or the impairment to perform rapidly alternating movements indicative of cerebellar dysfunction.

The Romberg test is often mistaken to be a test for cerebellar function, but it is actually a test of proprioception (dorsal columns). This test is done by asking the patient to stand with his arms outstretched forward. He must close his eyes and rely on proprioception to keep his body erect and balanced (without any visual information). He can be gently pushed to see if he can maintain balance.

Sensory. This can be assessed in an older child by pinprick, light touch, position, and vibration sense. Object discrimination, which tests for higher cortical functions, can be done using coins, paper clips, or rubber bands.

Reflexes. The segmental levels of major deep tendon reflexes are: Jaw jerk (CN V), biceps (C5-6), triceps (C6-8), brachioradialis (also known as the radial periosteal, C5-6), patellar (also known as quadriceps, L2-4), ankle (also known as Achilles, S1-2). The best known sign of pyramidal tract dysfunction is the Babinski's sign. This sign can be elicited when the plantar surface of the foot is stimulated with a stiff object (e.g., key) from the heel through the lateral aspect of the foot, crossing over the distal metatarsals, ending in the big toe. A positive Babinski's sign is seen when there is dorsiflexion (extension) of the great toe and fanning of the toes. This response can be normally seen in children up to 2 years of age or sometimes after a seizure. A positive Babinski's sign is also called an upgoing plantar reflex, as opposed to the normal downgoing plantar reflex which is called a negative Babinski's sign. Another sign is clonus that can be tested by maintaining dorsiflexion of the foot. Sustained clonus is abnormal at all ages and signifies a lesion in the pyramidal tract or the cortical origin of the pyramidal tract.

Neurological examination of the infant. The neurological examination of the infant can be organized in the following fashion: 1) Posture and muscle tone, 2) Primitive reflexes, 3) Age invariable items.

Posture and muscle tone. This can be divided in three ways: 1) resting posture, 2) passive tone, and 3) active tone. Resting posture can be performed by observing the infant undressed. The infant should have flexion of the elbows, hips, and knees (varying with age). Hypertonia in the extremities decreases after 3 months of age, with the upper extremities then the lower extremities. At the same time, tone in the trunk and neck increases. Passive tone is done by determining resistance of passive movements of the joints while the infant is awake and not crying. One can do this by flapping the hands and feet, and by other maneuvers. The scarf sign is where the arm is pulled across the chest and if the elbow passes the midline, then hypotonia is present. Active tone can be assessed by the traction response up to 3 months of age. The infant's hands are held with the examiner's thumbs in the infant's palms, and the fingers around the wrists. The infant is slowly pulled to a sitting position. Normally the elbows flex and the neck raises the head. If hypotonia is present, then the head lags backward, then as the erect position is assumed, the head then drops forward. If hypertonia is present, the head is maintained backwards.

Primitive reflexes. Primitive reflexes are usually present from the time of birth and represents spinal reflexes until the infant becomes older and higher cortical functions suppress them. Although there are many types of reflexes, it would be a good idea to do some of them and not necessarily all since they would not give more information than what was already done.

Vertical suspension. The infant is suspended by holding the chest with both hands and lifting the patient in an upright position, with the legs dangling. If scissoring or hyperextension of the legs is seen, then spasticity is present. If there is scissoring of the legs, then spasticity may be present making it suspicious that cerebral palsy may be present.

Horizontal suspension (Landau reflex). To perform this reflex, the infant, while prone with the examiner's hand under the trunk, is gently lifted upwards. Normally, the spine extends a little so that the eyes are looking just below the horizontal. If the body collapses into an upside down "U" shape, then hypotonia is present.

Segmental medullary reflexes. An example is the sucking reflex that involves afferent fibers of CN V and IX, and efferent fibers of CN VII, IX, and XII.

Moro reflex. This is done by having the head hyperextended, falling back about 3 centimeters in relation to the trunk. A normal response is seen when the infant opens his hands, extends and abducts the arms, and then brings them together, followed by a cry. It is present in all newborns and disappears before the age of 6 months.

Tonic neck response. Also called the fencer's stance. This reflex can be elicited when the head is turned to the side while the rest of the body lies flat on the table. A normal response is extension of the arm and leg on the side that the head is turned, and flexion of the arm and leg on the opposite side (similar to a fencing stance). Abnormal responses occur when this response is sustained or if it occurs differently when the head is turned to the right or left (i.e., the response is not the same when tested on both sides). It usually disappears about 6 to 7 months of age.

Palmar and plantar grasp reflexes. They are performed by applying gentle pressure to the palm or sole. An abnormal response occurs when this response is absent before 2 to 3 months of age, persistence after this time, or asymmetry.

Parachute response. The infant is suspended horizontally with the face down, and is brought quickly down toward the floor, making sure that the infant is firmly held. A normal response is seen when the arms are extended and the hands open.

Reflex placing and stepping responses. Reflex placing is seen when the dorsum of the foot is placed against the edge of the examination table. Reflex stepping is seen when the sole of the foot is placed on the table, and the infant appears to be walking. This reflex disappears at about 4 to 5 months of age.

Questions

1. Name the steps involved of the older child's neurological examination.

2. Name five primitive reflexes.

3. What extraocular muscles are innervated by abducens and trochlear nerves?

4. What does optokinetic nystagmus signify? When can it be performed in an infant?

5. What is the pronator sign? What does it test for?

6. In what two instances can a positive Babinski's sign be seen in normal patients?

References

1. Haslam RHA. Chapter 600 - Neurologic Evaluation. In: Behrman RE, et al (eds). Nelson Textbook of Pediatrics, 16th edition. 2000, Philadelphia: W.B. Saunders Company, pp. 1793-1802.

2. Menkes JH, Sarnat HB, and Moser FG. Introduction - Neurologic Examination of the Child and Infant. In: Menkes JH, Sarnat HB (eds). Child Neurology, sixth edition. 2000, Philadelphia: Lippincott Williams & Wilkins, pp. 1-32.

3. Inaba AS. Personal communication, 1999.

4. Tottori M. Personal communication, 2001. A black sheet paper is used and multiple strips of white tape (about 2 cm wide) are attached so that there are alternating strips of black and white. A photocopy of this is made and is then wrapped around an empty soda can. A straight piece of metal, such as from a dressing hanger, is used to pierce the top and bottom parts of the can and is thus the handle to rotate the drum.

I would like to thank Dr. Yoshio Futatsugi for his review of this chapter for the 1st edition. His assistance is greatly appreciated.

Answers to questions

1. Examination of the skull, cranial nerves, strength, cerebellar function, sensory, and reflexes.

2. Ventral suspension, horizontal suspension (Landau reflex), Moro reflex, tonic neck response (fencer's stance), palmar and plantar grasp reflexes, parachute response, reflex placing and stepping responses.

3. Lateral rectus and superior oblique muscles, respectively.

4. Signifies that cortical vision is intact, in addition to showing the integrity of the frontal and parietal lobes, and visual fields. It can be performed at about 4 to 6 months of age.

5. When the arms are lifted, a positive sign is when an arm is hyperpronated with the elbow flexed. It tests for strength of the upper extremities, and a positive sign signifies weakness.

6. In what two instances can a positive Babinski's sign be seen in normal patients? In newborns up to 2-1/2 years of age and sometimes in patients just after a febrile seizure.