Neuroscience Clerkship

 

 

 

THE NEUROLOGICAL EXAMINATION OF INFANT AND CHILD

 

Neurologic Examination of the Pediatric Patient

Introduction: The neurologic examination of the infant/child is very similar to that of the adult with one very important exception. It is much harder!! You have to rely on your powers of observation as the child usually can not tell you what the problem is. Symmetry vs. asymmetry is very important in the pediatric neurology examination.

Basic Components of the Pediatric Neurological Examination
Chief complaint, history, mental status, cranial nerves, motor, sensory, coordination, gait

Chief complaint: What is the patient’s or parent’s perception of the problem that brought them in to see you today. You may be surprised at the answers you get if you remember to ask this question at the beginning of your encounter.
History: Make sure to include all components of the history as described below.

History of present illness. Describe chronology in terms of “days prior to this visit”. Descriptors such as “2 weeks ago” are unhelpful to others reviewing your history at a later date. How long have they had this problem? Is it something they have noticed or was it noticed/brought to their attention by their doctor, school etc. Has it ever happened before? What evaluations have they already had for this problem. What treatments have they tried (you know, the “what makes it better, what makes it worse”)
 

Birth History
 

1) Prenatal history: Duration of the pregnancy, maternal infection, maternal use of medications, drugs including alcohol, tobacco, toxemia, vaginal bleeding, maternal disease such as diabetes, thyroid, myasthenia gravis, maternal PKU, quality/quantity of fetal movement during the pregnancy, product of multiple gestation pregnancy
2) Birth: C-section? Why?, hx of fetal distress, apgars, prolonged rupture of membranes, maternal fever, duration of labor, did the infant required resusitation? Apgar scores? Growth parameters at birth.
3) Perinatal/neonatal: Neonatal jaundice, neonatal fever, cyanosis, hypotonia, apnea, seizures, length of hospital stay

Developmental history: Be sure to ask the age at which skills are acquired. Any loss of milestones, slowing of development or regression. Review of all developmental areas is critical: Motor (gross and fine) Social, Language, Cognitive. Early onset handedness: hand preference develops at around 18months. Children that are “right handed” since birth may have a left hemiparesis!
“Potty training” is an important milestone. Loss of bowel or bladder control may signify seizures, spinal cord disorders such as tethered cord syndrome or mass.

Review of systems: It’s true what they told you in medical school, this really is important. It’s your chance to find out what other symptoms they might be having.

Go through the standard organ systems. In addition, questions important for neurologic history include: Have their been frequent hospitalizations for dehydration or feeding intolerance. Lead exposure? Head trauma?

Family History
-seizures
muscular dystrophy (age of presentation of relatives, level of severity
-consanguinity
-frequent spontaneous abortions in mother and first degree relatives (ask sex of fetus)
-blood disorders
-abundant birth marks

Examination

OBSERVATION, GENERAL MEDICAL EXAMINATION, MENTAL STATUS, CRANIAL NERVES, MOTOR, REFLEXES, SENSORY, CEREBELLAR, GAIT

OBSERVATION
Watch the child during your history. Observe him during play/interactions with family and surroundings. Look for any hyperactivity, any asymmetry to movements, excessive irritability, lethargy

GENERAL EXAMINATION

You should do a complete general examination of the child.
HEENT/HEART/LUNGS/ABDOMEN/GU/EXTREMITIES

HEAD

RECORD GROWTH PARAMETERS!!!!!!!!!!!!!!!!!!!!!!!!!!!!! YOU MUST MEASURE HEAD CIRCUMFERENCE ON ALL YOUR PATIENTS!!!!!!!!!!!!!!!!!

Mean HC at birth is 35 cm. Compare all growth parameters as microcephaly in the context of a normal height and weight is obviously more concerning. Head grows 2 cm in the first month, 6 cm by 4 months and 12 cm by the first year. If you suspect microcephaly/macrocephaly always measure the parents and siblings head.
-Ascultation of the head may reveal bruits.

Examination of the anterior fontanelle is important.
-This is best done when infant is upright.
- Pulsations are normal.
-a soft, full fontanelle is acceptable when the baby is crying or lying down

Appearance
Dysmorphic Features
Birth marks
-hypopigmented macules: tuberous sclerosis
consider woods light (ultraviolet light examination of child and parents)
-hyperpigmented spots: neurofibromatosis
-linear nevi on scalp (may be associated with seizures)
Examine midline of neck and back looking for skin dimples
Check the palmar creases
Check ear position
Unusual body odors : may suggest metabolic disorders

Cranial Nerves
This is a difficult exam in the uncooperative child. Much of what you learn is from close observation of the child.

CN 1: Not always tested. May not be functional until 5-7 months. Babies who grimace after given a noxious stimulus probably had their trigeminal nerve stimulated

CN 2: Funduscopic exam in children is similar to playing a Nintendo game. Discs are normally pale in the infant and appear like optic atrophy in the adult. Retinal hemorrhages are seen in 1/3 vaginally delivered infants. Note whether infant can track objects. At 8 wks the infant should be able to fixate and follow. Look for optokinetic nystagmus as a way to check for fixation. To check visual fields in a child, show them a toy in their central vision and slowly bring in another toy from the periphery. Check the red reflex on #10 black of the ophthalmoscope.
CN 3,4,6: Note position of the eyes at rest. Loke for “setting sun sign” as a sign of increased intracranial pressure. Check eye movements in all four quadrants-try to get child to follow brightly colored toy. Upgaze is limited for the first six months of life

LR6 (SO4) 3 Lateral rectus is cranial nerve 6, superior oblique is cranial nerve 4, all the rest of the Extraocular muscles are cranial nerve 3

CN 5: Sucking- tests masseter strength.
Corneal reflex: sensory 5, motor 7
Jaw jerk- if hyperactive suggests upper motor neuron lesion

CN 7: Look for symmetry upon smiling and crying. Rooting reflex
UMN: only lower face
LMN: upper and lower face

CN 8: Ability to turn eyes to the direction of a sound (7-8 weeks)
Turning of eyes and head to sound (3-4 months)

CN 9: Asymmetry of palate, uvula. Palate sensation and gag

CN 10, 11: Excessive drooling, hoarse cry

CN 12: Note how strong the suck is. Observe for tongue fasiculations. The tongue deviates toward the paralyzed side. ( The normal function of the genioglossus is to push the tongue to the opposite side)

Motor System
Think BATS
BULK, ABNORMAL MOVEMENTS, TONE, STRENGTH

BULK
Look for any asymmetry in muscle bulk (hemiparesis, lower motor neuron palsy) vs a generalized decrease
( muscular dystrophy/anterior horn cell disease, nutritional)

Abnormal movements

Tremor, fasiculations, myoclonus

Tone

The normal resting tone of the full term infant is predominantly flexor. They rest with their arms and legs semi-flexed.
-premature infants are hypotonic in comparison
*look for extended legs at rest, history of “great head control” at birth as clues to hyPERtonicity
*look for frog-leg position of lower extremities, exaggerated “scarf sign” as clues to hyPOtonicity.
( In the scarf sign the baby’s arm is drawn medially across the chest noting the position of the elbow. If the elbow crosses the midline in the full term infant this is a sign of decreased tone.)

Hypotonic infant: think sepsis, drugs, hypoglycemia, chromosomal, cretinism, anterior horn cell disease, myopathy.
Strength
Grading of muscle strength

0: No muscle contraction
1: A “flicker” of muscle contraction
2: Active movement of a joint with gravity eliminated
3: Active movement of a joint against gravity
4: Active movement against gravity and against resistance
(weak of varying degree (4+/5, 4/5, 4-/5)
5: Normal

Hand preference develops at 18-24 months. Early hand preference suggests hemiparesis. Compare thumb size of two hands. An old hemiparesis usually has a smaller thumb on that side.

Test a child’s distal strength by offering a toy and noting the resistance to your pull. Test a child’s proximal muscle strength by asking them to stand up from a supine position with arms folded across their chest. If they use their hands to “walk up themselves”, this is an indication of proximal weakness (Gower’s sign)

Reflexes
Deep tendon reflexes are normally brisk in children even into adolescence. Looking for symmetry is the key.
Triceps reflex is usually not developed in the infant
4-6 beats of ankle clonus is acceptable up to two months of age
Crossed adductor reflex can be present up to one year
Extensor plantar reflexes (babinski sign) are acceptable up to the time the child learns to walk

Grading of reflexes

0: no response
1+ decreased response
2+ normal
3+ hyper
4+ hyper + clonus
Sensory exam
Difficult and unreliable in the infant
Primary sensory modalities are touch, pain, temp, vibration (with a 128 hz tuning fork)
Cortical (integrated sensory modalities) are stereognosis, graphesthesia, 2 point discrimination
Cerebellar
Finger to nose: show child a light and ask him to touch the light
Rapidly alternating movement-synkinesis (mirror movements of opposite hand) common up to preschool age

Gait
Look for hemiparetic gait, circumduction of the foot, clumsiness, wide-based nature to gait.
Ask child to walk on heels, toes and tandem walk (difficult for children before school age)

 

 

The basic principles in evaluation of an infant are similar to those of an older child or adult.

SPECIAL CONSIDERATIONS

• Reliance on parents and others for historical information and observations.

• Exercise flexibility in approach to the examination.

• Simple observation often yields more information than formal testing. Variations in the norm are age dependent.


PHYSICAL EXAMINATION

Clues to neurologic disease may be obtained from a general physical exam (see Table 1 - not a comprehensive list). Features of the neurologic examination requiring special attention in infancy include:


Spine

Look for midline defects, dimples, tufts of hair, hemangioma. Palpate for body defects.


Head

Size and shape. Serial measurements rather than an isolated reading are important. An increase in head circumference of a centimeter per month is normally observed during the first six months, and a centimeter every two months for the remaining six months of the first year. Head circumference is one of the best measures of brain development in the infant and young child (see head circumference charts for boys and girls).  By age three, the majority will have achieved 90% of adult head size.

• Also look for generalized asymmetry, frontal bossing, prominent occiput, localized bulging.

• Microcephaly (below 3%)

• Macrocephaly (above 98%)

• Scaphocephaly - prominent antero-posterior head growth

• Brachycephaly - shortened antero-posterior with an increase in width

• Palpation of sutures - overlap, ridging, or separations

• Anterior fontanel - Mean age for closure is one year (range 7 to 19 months).

• Normally, the fontanel is somewhat depressed and pulsates, and should preferably be assessed in the upright posture and in a child who is not struggling or crying.


Auscultation

Systolic bruits may be heard in 50% to 70% of normal children below six years of age. Significant if loud, extend through both systole and diastole or are symmetrical.


Transillumination

Useful procedure in diagnosing intracranial lesions. It reveals an abnormal amount of transmitted light, which can vary considerably under normal circumstances dependent on:

• Extracranial fluid (infiltrated scalp IV)

• Thickness of the scalp

• Quantity of hair

Normally, there is slightly increased transillumination in the frontotemporal regions. Complete - glowing of the head - suggests hydranencephaly: severe hydrocephalus. Localized or asymmetrical transillumination suggests porencephalic cyst, Dandy-Walker anomaly, subdural effusions, atrophy.


Cranial Nerves
.
See Table 4.


Motor

Observing spontaneous activity and/or child in play activity can often reveal deficits of movement and coordination. Motor function varies with age.


Reflexes (Developmental). See Table 2 and Table 3.


DEVELOPMENTAL MILESTONES

These can be divided into gross motor, fine motor, language, and social behavior.

Interpretation:

• Normal

• Delayed in all spheres

• Normal in some, retarded in others

• Loss of previously attained skills

Older Child: Inquire about school performance. See Table 5.


TABLE 1. Systemic Signs in Patients with Chronic Neurological Deficit

Skin Eczema Phenylketonuria
  Pellagra rash Hartnup disease
  Ash leaf depigmented spots

"Shagreen patches"

Tuberous sclerosis
  Café-au-lait spots Neurofibromatosis
     
Hair Blonde Phenylketonuria
  Fine and sparse Cretinism, homocystinuria
  White and brittle Argininosuccinicaciduria and Menkes kinky hair syndrome
     
Abnormal body size Dwarfism Turner's syndrome
Lowe's syndrome
Cystinosis
Hypophosphatasia
  Tall stature Homocystinuria
Klinefelter's syndrome
     
Eyes Cataracts Lowe's syndrome
  Buphthalmos Homocystinuria

Sturge-Weber

  Glaucoma, Ectopia lentis Homocystinuria
  Chorioretinitis Toxoplasmosis
Cytomegalovirus infection
Rubella
  Retinal exudates Tuberous sclerosis
  Macula degeneration Neural lipidoses
     
Hearing Nerve deafness Neonatal jaundice (kernicterus)
     
Abnormal odor Musty Phenylketonuria
  Maple syrup Branched-chain ketonuria
  Dried celery Methionine malabsorption
  Sweaty feet Isovaleric acidemia
     
Organomegaly Liver and spleen Tyrosinosis
Gaucher's disease
Niemann-Pick disease


 


TABLE 2. Developmental Reflexes

REFLEX

APPEARANCE AGE

DISAPPEARANCE AGE

Adductor spread of knee jerks Birth 7 to 8 months
Moro Birth 5 to 6 months
Palmar grasp Birth 6 months
Plantar grasp Birth 9 to 10 months
Rooting Birth 3 months
Tonic neck response Birth 5 to 6 months
Truncal incurvation Birth 1 to 2 months
Parachute 8 to 9 months Persists
Landau 10 months 24 months


 


Table 3. Developmental Reflexes

Moro Reflex: Sudden abduction of the arms, extension of the legs, and flexion of the hips when the position of the head is changed abruptly in relationship to the body. The Moro reflex is present in all normal, full term infants. It is an indicator of the symmetry and intactness of the nervous system. It diminishes during the first months of life and usually disappears by 4 to 5 months.

Tonic Neck Reflex: With the infant supine, turning the head to one side results in extension of the arm and leg on that side with flexion of the contralateral area (i.e., fencing posture). It is usually not present in the newborn but appears after 2 to 3 weeks. The reflex is most prominent during the second month of life and infants may assume it spontaneously. An obligate or persistent tonic neck reflex is abnormal.

Crossed Adductor Reflex: Contraction of both hip adductors when either knee jerk is elicited. The crossed adductor response usually disappears by 7 to 8 months, and persistence beyond that time is a sign of pyramidal tract dysfunction.

Ankle Clonus: 8 to 10 beats may be present in the normal newborn, but generally disappears by 2 months of age.

Neck Righting Reflex: With the infant supine, turning the head to one side causes the infant to turn his shoulders and trunk to the same side. It appears when the tonic neck reflex disappears (i.e., at 4 months), when the baby begins to roll over. All normal infants have a neck righting reflex by age 8 to 10 months, after which it becomes part of voluntary activity.

Hand grasp: An infant is able to reach and grasp with his whole hand by 4 to 5 months of age. Thumb and finger (pincer) grasp begins at 6 to 7 months and is present in normal infants by 1 year. Transferring objects from one hand to the other begins at 7 to 8 months. A strong preference to use one hand is abnormal prior to 1 year of age, when the first clear evidence of handedness appears.

Posture in Horizontal Suspension: A test of head control and motor function. At age 5 months, infants held horizontally (parallel to the floor) begin to arch their backs and hold their heads above the horizontal plane.

Posture in Vertical Suspension: Flexor during the first half-year of life. Persistent adduction or scissoring of the lower extremities is always abnormal and a sign of spasticity. In the standing position, a normal positive supporting reaction consists of the child briefly bearing some weight. A "too good" positive supporting reaction is often the earliest sign of spasticity. In atonic dysplegia, withdrawal of extremities and lack of a positive supporting reaction may be present.

Parachute Reflex: The infant is suspended horizontally then plunged downwards; the reflex consists of arm extension to "break the fall." It begins at 6 to 7 months and is well developed by 1 year. The parachute reflex is an excellent test of upper extremity pyramidal function, and if asymmetrical, may be a sign of hemiparesis.

 


TABLE 4. Cranial Nerve Examination in Infancy

CRANIAL NERVE FUNCTION TESTED BY
     
I Olfactory nerve and tract Smell Simple odors (vanilla, tobacco, etc.), change facial expression suggests recognition
     
II Optic nerve and retina Visual acuity and fields Colored ball or block in visual field
     
III Oculomotor

IV Trochlear nerve

VI Abducens nerve

Extraocular movements, pupil response, lid elevation Observation of tracking, Vestibular response (gently rotating the child)
     
V Trigeminal nerve Mastication

Facial sensation

History of chewing

Corneal reflex

     
VII Facial nerve Facial expression

Taste

Observation for symmetry
     
VIII Acoustic nerve

Auditory division

Vestibular division



Hearing

Spatial orientation



Tuning fork, musical toy, rotation
     
IX Glossopharyngeal nerve

X Vagus nerve

Swallowing, voice History, feeding, quality of cry, gag reflex
     
XI Spinal accessory nerve Elevate shoulder

turn head

Observation in upright and prone positions
     
XII Hypoglossal nerve Tongue movements Symmetry, protrusion, atrophy, fasciculations

Adapted from The Practice of Pediatric Neurology, Swaiman, K. and Wright, F.


 


TABLE 5. Child Development from Two Months to Two Years*

2 Months Hands predominantly fisted
Lifts head up for several seconds while prone
Startles to loud noise
Follows with eyes and head over 90° arc
Smiles responsively
Begins to vocalize single vowel sounds
3 Months Hands occasionally fisted
Lifts head up above body plane and holds position
Holds an object briefly when placed in hand
Turns head toward object, fixes and follows fully in all directions with eyes
Smiles and vocalizes when talked to
Watches own hands, stares at faces
Laughs
4 Months Hold head steady while in sitting position
Reaches for an object, grasps it, brings it to mouth
Turns head in direction of sound
Smiles spontaneously
5 to 6 Months Lifts head while supine
Rolls from prone to supine
Lifts head and neck up in prone position
No head lag
Transfers object from hand-to-hand
Babbles
Sits with support
Localizes direction of sound
7 to 8 Months Sits in tripod fashion without support
Stands briefly with support
Bangs object on a table
Reaches out for people
Mouths all objects
Says "da-da," "ba-ba"
9 to 10 Months Sits well without support, pulls self to sit
Stands holding on
Waves "bye-bye"
Drinks from a cup with assistance
11 to 12 Months Walks holding on
Pincer grasp
Two to four words with meaning
Creeps well
Assists in dressing
Understands a few simple commands
13 to 15 Months Walks by self - falls easily
Says several words, uses jargon
Scribbles with a crayon
Points to things wanted
18 Months Climbs stairs holding on, climbs up on a chair
Throws ball
Builds two to four block tower
Feeds self
Takes off clothes
Points to two to three body parts
Many intelligible words
24 Months Runs, walks up and down stairs alone (both feet per step)
Two to three word sentences
Turns single pages of a book
Builds tower of four to six blocks
Kicks ball
Uses pronouns "you," "me," "I"

*Represents the age at which the average child acquires the skill. Data from Gesell, A., and Amatruda, C.S.: Developmental Diagnosis, Paul C. Hoeber, New York 1956; Illingworth, R.S.: The Development of the Infant and Young Child, 5th ed., Williams and Wilkens, Baltimore, 1972.