The scoring system used for both assessments at term and in the following years is common to the three different evaluation tools described previously. Each item is coded on a three level scale:
- A score of 0 indicates a typical result for that age, within the normal range;
- A score of 1 indicates a moderately abnormal result for that age;
- A score of 2 indicates a result which is definitely abnormal.
A score of X is used to keep track of the finding (e.g, the presence of primitive reflexes during the second trimester), but also to indicate that the result cannot be interpreted as normal or abnormal at the age considered. Similarly, the estimated value of the angle (on which the score is based) when analyzing passive muscle tone in the limbs should be noted so as not to lose any data.
Finally, a gray shaded area appears for certain items in the 0-6 years evaluation sheet; it indicates that the item does not have to be tested for that particular age column (e.g. the comparison of slow and rapid dorsiflexion angles of the foot during the first trimester of life).
Scoring charts for the Amiel-Tison and Gosselin Neurological Assessment from birth to 6 years
The chart allows for the recording of results for 10 successive examinations during the first 6 years of life; this period is divided for evaluation purposes according to the cerebral maturation rate. Maturation progresses at such a rapid rate during the first year of life that the scoring standards change every three months. To account for the slower rate of maturation during the second year, the scoring system changes every six months. After the age of 2, cerebral maturation evolves at a much slower rate, thus scoring for this age category does not change, except for acquisitions of new motor skills. These acquisitions are presented and summarized under “Motor Developmental Milestones”.
The chart is comprised of four main sections:
- Page 1 recapitulates the series of examinations from 0 to 6 years. Each age group is numbered in Roman numerals from I to X and the appropriate column test is chosen according to the age at the time of the examination, according to the instructions given later in this text. The child’s life environment and any subsequent changes in his or her life should also be taken into consideration. If the examination is conducted under clearly unfavorable circumstances this should be noted under “comments”.
- Page 2 allows for the recording of growth parameters including head circumference, height and weight.
- Page 3 allows for the recording of health problems and the morphology of the skull and face.
- Page 4 outlines neurosensory functions, seizures, alertness, attention and excitability.
This section (page 5) allows to summarize the gross and fine motor acquisitions usually acquired in the first years of life (examinations I through VI).
Three age-dependent neurological sections: each of these sections is comprised of four pages and allows for the recording of results regarding passive tone, motor activity, reflexes and postural reactions.
- The 0-9 months section (pages 6-9) is comprised of three examinations (I, II, III), which are conducted at 3-month intervals.
- The 9-24 months section (pages 10-13) includes the fourth examination of the first year of life (IV) and the two 6-month periods of the second year (V, VI).
- The section covering 2-6 years (pages 14-17) is for the examination conducted annually between 2 and 6 years (VII to X).
Section 4 The final profile summary sheet (page 18) includes:
- classification at 2 years based on the neuromotor and cranial signs together with the noted absence or presence of independent walking;
- evaluation of the motor function between 2 and 6 years and classification of cerebral palsy if applicable;
- disability in other areas of brain function;
- presence of epilepsy;
- extra-neurological conditions;
- social and family context.
Choosing the appropriate column
Roman numerals from I to X appear at the top of each column and correspond to the current month or year. For each evaluation, one of the columns numbered I to X will be chosen depending on the age of the child; for example, column I will be used for a child aged from 0 to 3 months, that is to say in the first, second and third months of life. At the end of the third month, the next column will be selected as the child begins his or her fourth month.
The entire evaluation procedure takes 10 to 15 minutes, sometimes longer if the child resists maneuvers or if the anomalies are particularly complex. There is no specific order for conducting this neurological examination. An observation or maneuver can be repeated if an adequate response is not obtained; in this case, the best response will be scored. It is recommended that the clinician writes down all data on the chart after completing the examination.
Minimal conditions for assessment In order for the result to be valid, one must:
- obtain a calm state that allows muscle release;
- make sure that the child’s head is in the midline to avoid interference with the asymmetric tonic neck reflex (see below);
- immobilize other joints when extensibility is sought on a poly-articular muscle (e.g popliteal angle with knees fixed laterally on each side of the pelvis);
- control his/her strength and know when to stop stretching as the child’s discomfort becomes noticeable.
Description of each maneuver/observation
Each maneuver or observation is described in a technical repertoire; typical or deviant results are analyzed according to the child’s age and include required instructions for scoring. This repertoire is the same as the one presented in the chart, with the description of a more general section followed by the neurological examination.
The growth parameters including head circumference, height and weight are measured. Proportionality or disproportionality taking into account the ratio between head circumference and the height is noted. At the end of the second year of life, the head growth profile can be defined on the basis of repeated measures. At each examination, the clinician will search for a number of health problems that can have impact on the neurological status and/or interfere with the neurological assessment.
Morphology of the skull and face
Examination of the skull by palpation is an integral component of the neurological examination in the early years; measurements alone (centimeters) are not enough to identify abnormal growth of the cerebral hemispheres.
If a ventriculo-peritoneal shunt was inserted as palliative treatment of hydrocephalus, its presence is indicated by an X, whether the shunt is still functioning or not.
The skull at this age is composed of small separate bones and it normally follows passively the significant volumetric brain expansion, because the fontanels are still open and sutures are still not fused. The surface area of each of the flat bones forming the dome of the skull expands outward from the central ossification area while the sutures between the individual bones remain edge-to-edge. But if brain growth is insufficient, overlapping due to premature fusing in cranial sutures and early closure of fontanels may occur. Such signs are therefore often associated with cerebral atrophy, even mild atrophy. As for the anterior fontanel, its size is highly variable so it is of no clinical interest here. Examiners should only be concerned with its status, i.e. open or closed. The normal variation is such that this criterion has little interest.
- 0 if the fontanel is open;
- 2 if the fontanel has closed prematurely, before 6 months;
- X if the fontanel is open or closed between 6 and 18 months.
Examination of each cranial suture is done by palpation: this includes the parieto-temporal suture situated above the auricle (also called the squamous suture due to its beveled edge shape), as well as the frontal, coronal, sagittal and occipital sutures.
- 0 if the bones are edge-to-edge and the sutures are therefore barely palpable;
- 1 if a ridge is detectable due to overlapping of the bones.
Shape of the skull
Some skull deformities immediately suggest the existence of a malformation syndrome. In the case of cerebral atrophy of fetal origin, the most common abnormality found with hypoxic-ischemic lesions is a narrow receding forehead (frontal bone), making the arch of the eyebrows seem to protrude abnormally and the ears to be abnormally large. In these cases, the HC itself is discordant with somatic growth (asymmetry to the disadvantage of the HC); the sutures overlap due to the lack of brain expansion. For this evaluation, only the obvious deformities are scored (in particular, frequent postural plagiocephaly).
- 0 if the shape of the skull appears normal;
- 1 if the shape of the skull appears abnormal.
When deformities are present, the examiner will detail their features in the chart, including the reasoning of their orientation value towards a specific etiology.
The tongue of the fetus is a large and powerful muscle, participating in the rhythmic activity of swallowing amniotic fluid. Palate shape can thus provide information on fetal brain function. Effectively, the lateral palatine ridges disappear during the last 3 months of pregnancy due to the exerted pressure created by the motor activity of the tongue during fetal life. This physiological deletion thus produces the flat palate normally seen in infants born at term. A high-arched palate may result from the non-deletion of lateral ridges due to insufficient molding forces by the tongue, of neurological origin. When this anomaly is present at birth in the context of labor at term, it strongly suggests a prenatal neurological injury associated with other neurological and cranial signs.
Fasciculations of the tongue
Fasciculations of the tongue are contractions of a group of muscular fibers visible in the form of a slight tremor at the surface of the tongue. To be accounted for in the assessment, fascicular movements have to be observed at rest and at the periphery of the tongue. This is an indication of a lesion in the nucleus of the 12th cranial nerve and therefore a sign that the brain stem has been affected.
Abnormal movements of the mouth and tongue These are involuntary movements of opening and distortion of the mouth. The tongue is often involved with protrusion movements that disturb the feeding process.
- 0 if absent;
- 2 if present.
Only results from clinical assessments are given a score. If there is any doubt or hesitation regarding findings, or as part of a systematic routine, specialized investigations should be conducted to confirm clinical assessments.
During the neonatal period, systematic assessments are conducted to check for hearing loss. This is done by observing the responses of newborns to acoustic stimuli such as white noise emitted at a level of 80 to 90 decibels. The percentage of false positives and false negatives however is quite high. Otoacoustic emissions (OAe) and brain auditory evoked potential (BaeP) are more reliable and tend to be tested systematically.
From 4 months of age, infants are tested with a series of acoustic toys (rattles, clickers, bells, music boxes). Moatti’s 4 boxes have the advantage of covering the necessary hearing scope for comprehension of speech and emitting 60 decibels of calibrated noise at 2 meters.
At approximately 9 months of age, the probability of deafness becomes stronger as evidenced by signs reported by the infant’s parents such as poor vocal sounds, little response when the child’s name is whispered or when noise is produced by acoustic toys. When responses are unclear or absent, additional tests are necessary.
- 0 if responses are within normal limits;
- 1 if hearing loss is moderate;
- 2 if hearing loss is severe
The objects used to elicit “fix and track” responses vary with age. During the neonatal period, examiners use a “bull’s eye” (glossy black and white concentric circles). After the first month, examiners use the Fantz target (black and white face) or their own faces to attract the infant’s attention. At approximately 6 months, smaller objects are used, such as pellets.
- 0 if “fix and track” responses are present in the first month and easily obtained due to a good level of alertness;
- 1 if “fix and track” responses are difficult to obtain and maintain;
- 2 if the infant shows no “fix and track” responses.
The results of further investigations are entered in light gray (visual evoked potentials (ENP), electro-retinogram (ERG), others). Nystagmus Nystagmus (whether horizontal, vertical or rotatory) must be evaluated (a few jerky lateral eye movements are not taken into account). It may be the result of non-fixation or be part of a neurosensorial syndrome. Further investigation is needed if this condition is observed.
- 0 if absent;
- 2 if present.
Eye movements Erratic eye movements indicate the absence of fixation.
- 0 if eye movements are coordinated;
- 2 if eye movements are erratic.
Strabismus results from a failure to align the visual axes, whether convergent or divergent, unilateral or bilateral. Synchronous eye movements are analyzed using the “fix and track” assessment. When strabismus is not immediately obvious but masked by binocular fixation, a systematic assessment is necessary to detect asymmetry of the right-left red reflections. The screen test will confirm this first impression and the test can then be systematically validated by an ophthalmologist.
- 0 if absent;
- 1 if present, whether constant or intermittent;
- X for variable strabismus before 6 months.
The “sunset sign” results from the downward rotation of the eyeballs; this is an indication of a brainstem lesion. The iris is partially covered by the lower eyelid and the sclera is visible above the iris.
- 0 if absent;
- 2 if present, whether constant or intermittent.
Behavior during the examination and neurosensory functions
During the first week of life, the spontaneous sleep-waking rhythm is difficult to judge; the child is often disturbed, the beginning of the feeding process is disrupting; his sleep periods are physiologically short, about 50 minutes interrupted by waking periods of about ten minutes. These periods lengthen and add up to approximately 2 hours of continuous sleep and waking periods of up to 20 to 30 minutes at the end of the first week. How to use these elements of judgment during a routine neurological assessment: by identifying the state the child is in at the beginning of the examination; by considering the child’s response to calming maneuvers if he was in a state 5, or to stimulating maneuvers if he was in a state 1 or 2; by maintaining as long as necessary the calm alertness state (state 3) which is the one allowing for the best neurological answers; by observing the smooth transition from one state to another or otherwise slowness, difficulty, failure; and by asking the mother and the nurse about the sleep-wake rhythm of the child. Within 2 hours after birth, the situation is often very favorable to test the vigilance because the newborn at term stays awake without crying, making the child available for this type of observation (a few hours later, and often during the first 2 days, he or she will be very difficult to wake up).
This prolonged state of quiet alertness during the first 2 hours after vaginal birth at term is extremely remarkable; it is advisable to take a few minutes for the exam, but also to allow the parents to enjoy their moment.
This state is related to the fetus’s response to the difficult passage, dilatation and expulsion leading to extra-uterine life. Catecholamines and endorphins create this particular state: the newborn looks around in his or her environment and easily demonstrates fix and track. It has been suggested that this physiological phenomena occurs to promote the mother-child bonding, considering how intense the interaction is during the first hours of life. Sometimes, however, the newborn is very excitable. There might be different causes: hypoglycemia, a fracture (especially a clavicle), or a painful lesion of the skull or face. Sometimes the newborn is lethargic and does not respond well to solicitations; this state may result from maternal anesthesia and needs to be discussed with the anesthetist. If vigilance is considered abnormal and results in poor social interactions during examination, the examiner should reschedule and see the child again. Often this state is transitory and alertness returns back to normal. Other times it is insufficient or becomes more serious and is associated with other neurological signs (see below).
A normal cry is ample, frank, varied, well-tolerated (from a cardiorespiratory viewpoint), and consolable. A pathological cry can be very high-pitched, excessive, monotonous, poorly tolerated and inconsolable; it can also be too rare, discontinuous, weak, or whining-like. The crying pattern is therefore a red flag for neurological or extraneurological injuries.
The definition of hyperexcitability in the newborn is not very precise, rather subjective, made of a set of signs that seem to deviate from the common neonatal behavior (because all newborns have periods of hyperexcitability, but they are brief and can be calmed). The signs are as follows: insufficient sleep, excessive inconsolable crying, frequent startling, tremors and clonic movements of the extremities or the chin (see below for definitions). On the contrary, hyperactivity can be identified based on excessive sleep, inability to obtain state 3 even with vigorous stimulations, lack of interaction with the environment, shortfall or lack of spontaneous motor activity. This state can be defined as lethargic. When this lack of interaction and spontaneous activity is extreme, one can speak of coma.
Alertness and attention
The quality of alertness and attention is determined by a rough estimate based on information from the mother and on observation of the child’s behavior during the examination. During the first few months, visual tracking is the most objective method for evaluating alertness and attention; receptiveness, sociability, smiles and vocal sounds are other elements of this subjective evaluation. As the child grows, assessment of alertness and attention becomes more approximate; most often, the child will be judged by communication and play during the examination sessions. Standard questionnaires completed by the parents can contribute further details (Mahone, 2005). The results of these questionnaires may be included in the summary profile.
- 0 if alertness and attention are normal for the child’s age;
- 1 for a moderate deficit judged by an excessive need for stimulation to obtain sustained participation;
- 2 for a severe deficit judged by an almost complete lack of participation despite prolonged encouragement; the mother can confirm if this lack of alertness is frequently observed or is the result of unusual circumstances.
The mother’s comments as well as findings during the neurological examination itself will allow the following symptoms to be identified: insufficient sleep, excessive inconsolable crying, frequent startling, tremors and clonic movements. These signs are usually grouped together to define excessive excitability.
- 0 if signs are absent;
- 1 if signs are compatible with normal life;
- 2 if the condition is uncontrollable by usual means.
Seizures and benign myoclonus during sleep
Different types of seizures will not be described here; whether generalized or focal, the clinical aspects are extremely varied. Parents have difficulty recognizing seizures, particularly in children who are the most seriously affected. Examiners must therefore ask parents simple and straightforward questions about each episode such as:
- Who was the child with when the suspicious symptoms occurred?
- What time of the day or night was it? Was the child awake or asleep?
- What were the observed symptoms? Were they simultaneous or one after the other?
- Was the child able to respond to you? Were the child’s eyes staring straight ahead or turned upwards or to one side, right or left? Was the child stiff or limp in your arms? Were the child’s limbs shaking? On both sides or just one?
- What did you notice after the crisis? How long did that last? Was the child tired? Was he/she walking normally? Did he/she fall down a lot? Any vomiting?
Seizures that occur when the child has a fever – called febrile seizures – do not have the same significance as seizures that have no apparent cause. For this reason, febrile seizures are not recorded on the chart. If the child’s temperature is 38° C or higher during the episode, it is indicated with an X.
- 0 if seizures are absent;
- 1 for focal or easily controlled seizures;
- 2 for severe, prolonged and repeated seizures;
- X for febrile seizures.
For clinical description of seizures and myoclonus during sleep Motor milestones
Normal cerebral maturation enables a schedule to be established for neuromotor acquisitions, including average age and acceptable time limits. Each of these motor development milestones reached “on time” is given a score of 0 and the exact age of the child is indicated. If a delay is observed, the result is considered as moderately abnormal and given a score of 1. If a more clearly defined delay or the absence of a neuromotor acquisition is observed, a score of 2 is recorded (see time limits for each acquisition below). If any abnormality is present, the examination of the neuromotor function may be extended using specific measuring instruments.
Gross motor milestones
The gross motor development milestones in the context of this basic assessment are limited to three: control of the head, sitting and walking. Other stages such as turning over, crawling, or all-fours are not analyzed here, although they are all interesting skills to observe.
For simplification reasons, the stages of maturation leading to head control have not been analyzed in this section of the chart. The child’s ability to hold his/her head steady is the first motor acquisition, which results from the synergic activity of flexor and extensor muscles. This demonstrates the integrity of the infant’s upper cerebral control.
Technique: The infant is held in a sitting position. If the head is held steady in the axis of the trunk for at least 15 seconds, head control is present. The average age of acquisition is 2 months.
- 0 if present before 4 months;
- 1 if acquired during the 5th or 6th month;
- 2 if acquired after 6 months or absent after 6 months.
Sitting independently is the result of previous stages (leaning forward on arms, as at five months, then without arm support). Independent sitting occurs when the child sits alone for 15 seconds or more without using his or her arms to maintain the posture. The average age of acquisition is 7 months.
- 0 if acquired before 9 months;
- 1 if acquired between the 10th and the 12th month;
- 2 if acquired or absent after 12 months.
Walking independently Independent walking is achieved when the child can take at least three steps unaided. The average age of acquisition is 13 months.
- 0 if acquired before 18 months;
- 1 if acquired between the 19th and 24th month;
- 2 if acquired or absent after 2 years.
Fine motor milestones Putting a cube into a cup (by imitation) The voluntary release of a cube into a cup with a 10-12 cm opening is associated with the gradual disappearance of the grasping reflex. Technique: With the child seated, the examiner places a 2.5 cm cube and a cup in front of him/her then demonstrates the activity. The child drops the cube into the cup; the wrist should be slightly extended and the child’s arm should not lean on the cup for support.
- 0 if acquired before 10 months;
- 1 if acquired between the 11th and 14th month;
- 2 if acquired or absent after 14 months.
Grasping a pellet
Picking up a pellet with the thumb-index pinch requires proper functioning of the corticospinal system. This pinch implies dissociation of finger movements and opposition of the thumb.
Technique: With the child seated, the examiner places a small 0.5 cm object (such as a raisin) on the table in front of the child. He or she grasps the object using thumb-index finger opposition. Slight flexion of the thumb is observed at the metacarpophalangeal joint and more pronounced flexion is observed at the interphalangeal joint. The child should use the terminal or subterminal surfaces of the thumb and index finger to grasp the object.
- 0 if acquired before 12 months;
- 1 if acquired between the 13th and 15th month;
- 2 if acquired or absent after 15 months.
Building a three-cube tower (by imitation) Building a tower using cubes requires good visual motor skills and adequate postural control. Technique: The child is seated at a table adjusted to his or her size; five 2.5 cm cubes are placed on the table in front of the child. The examiner demonstrates the activity once. The child should stack at least three cubes.
- 0 if acquired before 21 months;
- 1 if acquired between the 22nd and 24th month;
- 2 if acquired or absent after 2 years.
Double circle of index and thumb of both hands (by imitation)
This is one of the most commonly used tests based on the imitation of complex gestures (Bergès and Lézine, 1978). When done successfully it proves that the subject immediately perceives the pattern achieved by the person in front of him and is able to reproduce it without hesitation. The test can be offered to 4 year-olds but scoring can only start after age 5.
Technique: The examiner sits in front of the child and performs a complex movement with his/her fingers; two circles formed by the thumb and the index, leaving the other fingers lightly bent. He tells the child “Now do like I do with my fingers”. The example is held for 10 seconds. The child either reproduces the movement immediately (without hesitation), reproduces it after the pattern has been broken down, or he/she gives up and abandons the test. (A response which is hesitant or unsuccessful is scored from 5 years old onward).
- 0 in the case of immediate success;
- 1 in the case of a hesitant response at 5 years (attempts and errors, breakdown of the pattern);
- 2 in the case of failure at 5 years.
SECTION II Passive muscle tone Upper limbs Scarf sign maneuver
This maneuver evaluates the extensibility of the trapezius muscles, the abductors and the external rotators of the shoulder (the neck remains in position while the acormial insertion of the trapezius is moved). Technique: During the first few months of life, this maneuver is performed as follows: with his or her elbow resting on the examination table, the examiner supports the head and neck of the infant in a semi-supine position with one hand. One of the infant’s hands is taken and the arm is pulled across to the opposite shoulder as far as possible. The examiner then observes the position of the elbow in relation to the midline. Later, when the infant can sit independently, the maneuver can be performed in the sitting position, with the observer either behind or in front of the child. The infant’s head must be kept in the midline at any age.
Results: To help examiners with their observations, three elbow positions are defined:
- Position 1: does not reach the midline
- Position 2: passes the midline
- Position 3: reaches very far across with little resistance.
Scoring is age-dependent; see chart for recording a score of 0, 1 or 2.
- In the term infant, resistance to stretching is very strong and then gradually dissipates.
- At 2 months, the elbow almost reaches the midline (this is the first indication of the descending wave of relaxation when upper control is intact).
- At or before 9 months, there is little or no resistance and the arm can be wrapped around the neck.
Examiners should give a score only for a complete lack of resistance before 9 or after 18 months of age and not in between, as hypotonia is physiological at this stage of development.
Limb Flexion (for the examination at term)
When limbs are flexed, as normally observed in newborns at term, it is easy to check the strength of the flexion. The examiner extends the elbow or the knee and observes, he/she releases the distal segment, there is an abrupt return to the initial position of flexion. The examiner can repeat the maneuver to check whether or not the response is exhaustible; extension can be maintained for ten seconds to check whether or not the response is inhibited. Whatever the result, it is normally symmetrical.
Technique: With the child supine, the examiner extends the child’s legs and gently spreads them apart laterally, as far as possible. The angle formed by the two legs is the adductor angle. The extensibility of the left and right adductor muscles, evaluated simultaneously, is measured by the degree of this angle. Pattern of changes: The adductor angle gradually increases over the first 9 months: at 2 months, it will open up 40° to 80° and, at 9 months, 100° to 140° or more, with the lower limbs offering very little resistance. Between 9 and 18 months, the age of physiological hypotonia, the angle can be limitless. Over the following years, the adductor angle will slowly decrease until it reaches average adult measurements.
Scoring is age-dependent; see chart to record a score of 0, 1 or 2. Adductor angle values that fall below age standard measurements help examiners identify abnormal hypertonia of the adductor muscles. Examiners should record a complete lack of resistance between 9 and 18 months with an X. If detected before 9 months or after 18 months, a score of 2 should be recorded.
Technique: While keeping the child’s pelvis on the table, the examiner laterally flexes both thighs at the pelvis to each side of the abdomen. With the thighs held in this position, the legs are extended as far as possible. The angle formed by the leg and the thigh is the popliteal angle. The left and right angles are evaluated simultaneously. The measurement of this angle indicates the extensibility of the hamstring muscles.
Pattern of changes: The popliteal angle will gradually increase over the first 9 months. At 2 months, it will be approximately 90°; at 9 months, 120° to 150°. It can be limitless between 9 and 18 months (the age of physiological hypotonia). The popliteal angle will slowly decrease over the following years until it reaches average adult values.
Scoring is age-dependent (see chart to record a score of 0, 1 or 2). Popliteal angle values that fall below age standard measurements help examiners identify abnormal hypertonia or shortening of the hamstring muscles. A complete lack of resistance is recorded with an “X” between 9 and 18 months, because the existence of physiological hypotonia is often quite marked; it should be given a score of 2 before 9 months and after 18 months.
Dorsiflexion angle of the foot: slow maneuver
Technique: The examiner flexes the foot toward the leg by slowly pressing the plantar surface of the foot. The angle formed by the longitudinal axis of the foot and of the leg is the dorsiflexion angle. This maneuver is performed first on one foot and then on the other, with the examiner’s hand placed on the knee to keep the leg straight (this slow angle measures the extensibility of the triceps surae with the knee extended in order to include the gastrocnemius). The slow angle is the smallest angle obtained by applying light pressure. When the child is older, the examiner will take the whole foot, including the heel, in the palm of his/her hand to obtain the maximum stretching of the triceps surae.
- The first few months:The starting point varies according to gestational age at birth. In the full-term infant, the dorsiflexion angle of the foot is close to 0° since the in utero pressure during the last weeks of pregnancy gradually reduces the dorsiflexion angle of the foot. In the premature infant who has reached the age of 40 weeks, the angle remains wide, as it was at birth (e.g. 40° to 50° at 28 weeks). Later, during the first few months, the 0° angle of the term infant will gradually increase to match the angle of the premature infant. This is why this maneuver is only performed after the fourth month and only angles exceeding the normal upper limit are taken into account (more than 80°, gray area).
- Later development: If permanent dystonia is associated with spasticity detected in the triceps surae (see below), a permanent equine deformity of the foot may result, leading to the shortening of both the muscle and the tendon, thus making the slow angle excessively wide.
- 0 if the dorsiflexion angle is equal to or less than 80°;
- 1 if the dorsiflexion angle is between 90° and 100°;
- 2 if the dorsiflexion angle is equal to or greater than 110°.
Dorsiflexion angle of the foot: rapid maneuver
This “rapid angle”, as the dorsiflexion angle is so called, enables examiners to evaluate the spasticity of the triceps surae. Technique: The same dorsiflexion movement as in the slow maneuver is repeated more rapidly. Frequent repetition of the manipulation often makes an abnormal response more obvious.
- 0 if the dorsiflexion angle obtained with rapid stretching of the triceps surae is identical to the angle obtained with the slow maneuver;
- 1 in the case of phasic stretch;
- 2 in the case of tonic stretch.
As explained above, it is necessary to define wide standards for passive tone in the limbs due to individual variations. Asymmetrical findings noted during the examination will become evident when comparing the scores of the left and right sides of the body. However, the broad definition for normal range of passive tone made necessary by individual variations means that asymmetry can be present even when all the values (both left and right) have been given a score of 0. This asymmetry therefore represents an abnormality in itself, which is clearly visible and significant within the normal range (with the normal side as the control, individual variations disappear, and thus make it easier to detect a moderate abnormality when it is unilaterally predominant). Asymmetry can also be an important indication of a particular type of cerebral lesion. It can therefore become a valuable marker in the interpretation of data.
- 0 if asymmetry is absent or cannot be categorized;
- 1 if one side (left or right) is more tonic than the other.
Slow dorsal extension of the trunk evaluates the extensibility of the anterior axial muscles (all the pre-rachidian and abdominal muscles). Technique: With the infant lying on his or her side, the examiner grasps the lumbar region of the spine with one hand and, with the other hand, tries to push the pelvis and lower limbs backwards.
- 0 if dorsal curvature is absent, minimal or moderate;
- 2 if dorsal curvature is very marked, arcing easily (also known as opisthotonos).
Passive flexion of the trunk (ventral curvature) Slow ventral flexion of the trunk evaluates the extensibility of the posterior axial muscles (trapezius and all the paravertebral extensor muscles). Technique: With the infant lying on his or her back, the examiner grasps both the legs and the pelvis and pushes them toward the head to test the maximum curvature of the spine.
- 0 if ventral curvature is moderate but easy to obtain.
- 1 if ventral curvature is absent or minimal.
- 2 if ventral curvature is unlimited, indicating extreme hypotonia.
Comparison of dorsal and ventral curvatures of the trunk
It is impossible to define the extent of passive flexion and extension of the trunk precisely. Curvature is evaluated visually, not measured. Resistance to passive manipulation is felt through the examiner’s hands, not measured. Flexion and extension vary with age. Therefore, both values are greater during the period of physiological hypotonia. They also vary with articular and individual factors, as well as poor tolerance for manipulation in the supine position. It is by comparing both curvatures that examiners arrive at the best interpretation of passive tone of the axis and its deviations. Comparative scoring should be as follows at any age.
- 0 if flexion is greater than or equal to extension, i.e. a certain degree of anterior curvature and little or no dorsal extension (flexion and extension are given a score of 0);
- 1 if flexion is more limited than extension (flexion is scored 1 because it is minimal or absent, and extension is scored 0 or 2). This situation indicates a lack of upper control of the antigravity muscles;
- 2 if both curvatures are unlimited (both scored 2). Findings are interpreted as extreme axial hypotonia.
Active muscle tone and motor activity Induced motor activity: active tone (for the examination at term)
Motor activity can also be tested by maneuvers that provoke an active movement of the child; it is the active tone or induced motor activity. Some maneuvers allow us to explore the responses of the head and neck, while others provide insight into the control exerted on the antigravity forces (the lower system) by the upper system. The two most frequently used maneuvers are:
- the raise-to-sit and back to lying maneuver;
- the righting reaction.
Raise-to-sit and back to lying
This maneuver allows the comparison of flexor and extensor muscles of the head in a very precise analytic situation. The examiner shifts the trunk from the lying to the sitting position, then back to lying. He or she then observes the child’s reaction to this move. The movement of the trunk should be neither too fast nor too slow:
- if it is done too quickly, it is not possible to judge whether the child’s head follows the movement passively or if the neck muscles are actually active;
- if it is too slow, it does not trigger the response, which can be mistaken as absent. The optimal speed to trigger a well “readable” reaction is learned by experience.
For the first part of the maneuver, the examiner holds the infant’s shoulders and pulls him or her from the lying to the sitting position. The forward movement elicits active contraction of the neck flexor muscles (sternocléido-mastoid ) in an attempt to raise the head to a vertical position.
For the second part, the return back to lying, the infant is held in the sitting position with the head hanging forwards on the chest. The examiner moves the trunk gently backwards while observing the reaction of the head extensor muscles (especially the trapezius whose acromial insertion is secured by the observer’s hand) which elicit the active passage of the head backwards. In the infant born at term, gentle movements of the trunk around the vertical position evoke active and symmetrical movements, which show a perfect balance between the two sets of muscles and only minimal lag. Sometimes the responses are so great that it is difficult to break down and distinguish the observation between the two muscle groups. However, during the first weeks of life, when the child is held in a sitting position, the head cannot be held up in the axis for more than a few seconds: it oscillates, falls forward or tilts sideways. Control of the head appears around 2 months on average, the age at which the child can keep it in the axis for 30 seconds or more.
In testing the neck muscles, three distinct abnormalities can be detected:
- the overall deficit results in a dangling head, meaning that abnormal tone is detectable by the weakness or absence of both flexor and extensor responses;
- there is weakness or absence of flexor muscle activity; it is insufficient to lift the head forward;
- there is excessive action of the extensor muscles, which pull the head back too soon and too far when the trunk is moved backward from the sitting position.
Righting reaction (or straightening)
The examiner places the infant in the standing position, with the feet on a horizontal surface while supporting the trunk with one hand. Plantar cutaneous stimulation triggers a straightening antigravitic reaction. It is recommended to hold the child with one hand, placing the thumb and middle finger in each armpit, with the index close to hold the head in the case that is becomes necessary to provide support. This position allows to perceive how much body weight the child is able to support. There are two types of abnormal responses:
- absence of righting: it has little meaning in the first days because of the normally flexed position of lower limbs in utero;
- excessive contraction of the antigravity muscles with opisthotonos, suggesting very clear evidence of the predominance of extensor muscles (that is to say the sub-corticospinal system).
Face Facial expression
Observation of facial mimicking is part of the neurological examination. Facial expressions are normally greatly diversified and contribute to showing pleasure, pain and various discomforts.
- 0 if facial expressions appear normal, symmetrical and varied;
- 1 if the mimicking is poor, stereotyped, not expressive, asymmetrical.
Constant drooling is a sign similar to facial expression but it is too common before the age of one to be recorded as abnormal. After the age of 2, it becomes one of a series of severe signs.
- X if drooling is present or absent during the first year;
- 0 if it is absent during the second year;
- 1 if it is present during the second year;
- 2 if it persists significantly after 2 years.
Facial paralysis, whether unilateral or bilateral, is scored. The affected side is indicated and it is also noted whether it is central or peripheral in character. If the disorder is central, the controlateral facial nerve compensates the deficit in the upper part of the face and the eye therefore closes normally. If the disorder is peripheral, the paralysis is total and the eye remains open.
- 0 if facial paralysis is absent;
- 2 if facial paralysis is present.
Spontaneous motor activity
Spontaneous limb movements are observed in the supine and prone positions during the first few months of life. Later on, all gestures during play will be observed. Scoring is based on making a qualitative global assessment. Certain movements, such as the arms rolling in extension and pronation, are recognized as being highly abnormal and will therefore be indicated in the chart.
- 0 at all ages if limb movements are smooth and varied;
- 1 if limb movements are insufficient, uncoordinated or stereotypical;
- 2 if limb movements are barely present or very uncoordinated.
- 0 if an open hand and finger movements are observed; these movements are observed spontaneously when the infant is awake;
- 1 if the hands are constantly closed with no visible finger movements at examination I (conducted between the first and third month of life) even if the fingers can be spread apart easily;
- 2 if the hands are constantly closed from examination II onward, and circle 2 at any age if no movement of the thumb is observed and if it remains permanently adducted inside a fisted hand.
Primitive reflexes and postural reactions
The time it takes primitive reflexes to disappear varies substantially and this must be taken into account when scoring. For scoring purposes, their presence will only be considered abnormal after 6 months. After 9 months, evaluating primitive reflexes is no longer beneficial, with the exception of the asymmetric tonic neck reflex, which is often the only primitive reflex that shows obvious or subtle persistence in cases of moderate abnormality. All primitive reflexes are indicators of a lower system not fully controlled by an upper system. Consequently, they all have the same significance, because of their presence during the early months, then later, because of their absence. Testing of a few of these reflexes will be sufficient.
Primary reflexes Sucking
Technique: Non-nutritive sucking is easily analyzed by inserting the fifth finger curled downward on the middle part of the tongue. This contact alone stimulates the sucking reflex. Sucking is not a continuous phenomenon; it consists of bursts of movements separated by rest periods. In the term infant, there are usually 8 or more sucking movements in a burst, the rhythm is rapid and bursts last for 4 to 5 seconds. A strong negative pressure is felt (the examiner’s finger is strongly aspirated) when facial motor ability is normal, ensuring a good seal of the lips around the finger.
- 0 if characteristics of normal sucking are present (rhythmic movements with adequate negative pressure);
- 1 if the number of repetitions and negative pressure are insufficient;
- 2 if sucking is absent or completely inadequate (due to the lack of closure of the infant’s mouth around examiner’s finger or weak tongue movements).
Technique: With the child lying on his or her back, the examiner gently raises the child a few centimeters off the table by both hands, with the infant’s upper limbs in extension. When the examiner quickly lets go of the child’s hands, he or she falls back onto the examination table, and the Moro reflex appears. The first observation is abduction of the arms with extension of the forearms (arms open), followed by adduction of the arms and flexion of the forearms (arms embrace). During the first part of the reflex, the infant’s hands open completely. A cry and an anxious expression are part of the response.
- 0 if the first part of the reflex is present during the first 3 months;
- 2 if the reflex is present after 6 months;
- X if the reflex is present between 3 and 6 months.
Grasping reflex of the fingers
Technique: The examiner places his/her index fingers in the palm of the infant’s hands. This palmar stimulation causes strong flexion of the fingers. This maneuver, usually done on both hands at the same time, elicits the grasping reflex.
- 0 if the reflex is present during the first 3 months;
- 2 if the reflex is present after 6 months;
- X if the reflex is present between 3 and 6 months of age.
Automatic walking reflex
Technique: During the first few months, this maneuver is conducted as follows: the infant is held in a vertical position with one hand placed in the upper thoracic region, with the thumb and middle finger under each armpit (the index finger is kept free to prevent the head from moving too much, if necessary). After the first few months, the infant is held in a vertical position by placing one hand under each armpit. The examiner observes the straightening of the legs and trunk. The infant should support most of his or her own body weight for a few seconds. The infant is then gently tilted forward and should take a few steps.
- 0 if the reflex is present during the first 3 months;
- 2 if the reflex is present after 6 months;
- X if the reflex is present between 3 and 6 months.
Asymmetric tonic neck reflex (ATNR) (or fencing posture)
Technique: 1 – During the first few months, spontaneous asymmetric tonic neck reflex can be observed when the infant is lying in a dorsal decubitus position. If the infant’s head is rotated to either side, a particular posture of the limbs can be observed. In its spontaneous form, the infant will extend the upper limb on the side toward which his/her face is turned, i.e. facial side (increase in extensor tone) and flex the upper limb of the opposite side i.e. occipital side (increase in flexor tone). A similar but less prominent response may also be elicited in the lower limbs. When this posture is observed, it is called “evident ATNR” in the chart. Like other primitive reflexes, this fencing posture (facial arm extended and occipital arm flexed) is normal in premature and term newborns during the first few months of life. After the age of 6 months, evident ATNR is a sign of insufficient upper control over subcortical cerebral function. It remains present in severe cases of CP and seriously restricts voluntary motor activity.
Technique 2 – After 4 years, the subtle persistence of this posture is evaluated (before 4 years of age, this reflex is too difficult to elicit, as the child does not understand the instructions). The child is placed on hands and knees with arms extended. To avoid the elbows being locked in extension, the child’s hands are turned inward, with fingers facing each other. The examiner passively rotates the child’s head. Normally, this does not change the support of the arms and they remain extended. If flexing of the occipital arm is observed, the asymmetric tonic neck reflex is present but it will barely, if at all, interfere with voluntary motor activity and therefore does not have the same pejorative meaning as “present, evident”.
- 0 if the ATNR is absent at 6 months;
- 2 if the ATNR is markedly present after 6 months;
- 1 if, after the age of 4 years, flexing of the occipital arm is observed when the infant is on all fours (“present (elicited)”).
- X if the response is present or absent before 6 months.
Protective reactions appear during the first 12 months in response to a sudden displacement. Once they have appeared, these reactions persist throughout life. The absence of protective reactions is therefore interpreted according to age. The response on each side is noted in order to confirm asymmetries.
Normally, these protective responses precede the appearance of the function, sitting or walking. In the case of a severe disorder, these reactions are not present. In the case of a moderate or minor disorder, they appear later.
Lateral propping reaction (or static aptitude)
Technique: With the infant siting independently, the examiner abruptly pushes the child laterally at shoulder level. The child should extend his or her arm to the opposite side to avoid falling. This postural reaction is elicited only after a firm sitting position is acquired, normally between 6 and 8 months. It is therefore not tested during the first 6 months.
- 0 if this reaction is present from 6 months;
- 1 if this reaction is incomplete between 9 and 24 months;
- 2 if this reaction is absent after 9 months (even if the test cannot be performed due to the inability to sit independently) or if the response is incomplete after 24 months.
Parachute reaction (protective extensor thrust)
Technique: With the child held in a ventral suspension position against the examiner’s body, the child is suddenly pushed with the head forward toward the examination table. The infant should immediately extend the upper limbs with extension of fingers, as if to protect himself or herself from falling. The precise reaction only appears between 8 and 9 months. It is therefore not tested during the first 6 months.
- 0 if the response is present from 6 months;
- 1 if the response is incomplete between 9 and 24 months or absent between 9 and 12 months;
- 2 if the reaction is absent after 12 months or incomplete after 24 months;
- X if the response is incomplete or absent between 6 and 9 months.
Left-right asymmetry in primary reflexes and postural reactions
Asymmetry in the reflex response can be observed even within a response normally present for age (i.e. scored 0). In such a case, we note the deficient side, particularly noticeable in the Moro reflex and grasping reflex of the fingers.
Deep tendon reflexes
Deep tendon reflexes (DTR) are of little interest between 0 and 2 years, as hyper-excitability is physiological during this period. The responses are highly variable, as is the diffusion of responses. If diffused responses persist after 2 years, the phenomenon becomes more discriminative. A rough quantification system is used to preserve validity from one examiner to another. More useful than a refined quantification of the response itself, however, is the identification of left-right asymmetries. DTR are sought out, as much as possible, in an infant who is calm and relaxed. Only the patellar reflex is scored.
- 0 if the response consists of a few moderate movements;
- 11 if the response is very brisk and accompanied by clonic movements;
- 2 if the response is diffused to other muscle groups after age 2 or if the response is absent under adequate examination conditions;
- X for a brisk response with clonic movements and diffusion observed up to age 2.
Neuromotor abnormalities and acquired deformities
It is interesting to note certain anomalies prior to the acquisition of a function since they often indicate the most probable physiological mechanism. However, the frequent intertwining of CNS and peripheral mechanisms must be handled with caution when drawing conclusions from a single examination.
Shortening of the trapezius muscles can be due to prolonged abnormal postures in the intensive care unit. It is also maintained by almost exclusive use of the supine position without particular attention being paid to the upper extremities. Muscular shortening is caused by the medial and external insertions (clavicle, acromion and the upper ridge of the scapula) being closer to one another. When the child is observed from behind in a sitting or supine position, the arms are externally rotated and the forearms are flexed in such a way that the upper limbs form a two-armed candlestick.
- 0 if this posture is absent;
- X if this posture is present.
Head control Holding the head behind the axis
This commonly occurs during the first 6 months. The head does not fall forward when the trunk is leaning forward and the chin pointing upward in a typical chin-forward position. The examiner can feel that the trapezius muscles are short and stiff. This false control of the head can be the result of hypertonia of the extensors (a central nervous system problem) or a shortening of the trapezius muscles due to abnormal postures. Because the interpretation is uncertain, head control behind the axis will not be scored before 6 months of age.
- 0 if this abnormality is absent;
- 2 if this abnormality is present;
- X if this abnormality is present before 6 months.
Poorly maintained head control due to fatigue.
This is an indicator of partial disability affecting both the flexors and the extensors. The child can voluntarily lift his or her head on request but can only support it for a relatively short time due to fatigue or lack of attention. This abnormality is not scored before 6 months of age.
- 0 if the abnormality is absent;
- 2 if the abnormality is present;
- X if the abnormality is present before 6 months.
Sitting position Failure to sit up due to falling forward
This is very common in the process of acquiring independent sitting. Falling forward is a result of a certain degree of axial hypotonia. Because it is a simple exaggeration of a physiological phenomenon, it is not scored until 9 months.
- 0 if this abnormality is absent;
- 1 if this abnormality is present between 9 and 12 months;
- 2 if this abnormality is present after 12 months;
- X if this abnormality is present between 6 and 9 months.
Failure to sit up due to falling backward
This is caused partly by the inability to adopt the tripod position (because hypertonia of the flexor and adductor muscles in the legs keep the knees too high and close to one another), and partly by imbalanced axial tone in favor of the extensor muscles. Falling backward is therefore unavoidable.
The presence of this abnormality is scored from 6 months of age; it is not due to an exaggeration of a physiological phenomenon but to an excess of antigravity muscle tone.
- 0 if this abnormality is absent;
- 1 if this abnormality is present between 6 and 12 months;
- 2 if this abnormality is present after 12 months.
Falling backward is very often observed in cases of spasticity since it is an indicator of insufficient upper control in axial tone. It is often associated with an arching response to standing on the floor due to the same mechanism. Poorly maintained sitting position due to fatigue This observation has the same significance as that indicated for head control. It is scored after 1 year of age.
- 0 if this abnormality is absent;
- 2 if this abnormality is present after 12 months;
- X if this abnormality is present before 12 months.
Standing position Excessive extension in standing
This is an excessive reaction of the antigravity muscles when standing on the floor which creates an opisthotonos (or arched) posture. Excessive extension in standing is abnormal at any age.
- 0 if this abnormality is absent;
- 2 if this abnormality is present.
Acquired axial deformities
This refers to a common complication of cerebral palsy in cases of unilateral or predominantly unilateral lesions, according to the distribution of paralysis, spasticity, and orthopedic problems in the pelvic region and lower limbs. This deformity will be scored after 2 years of age.
- 0 if this deformity is absent;
- 1 if this deformity is present from 2 years.
This is a common deformity caused by spasticity and shortening of the hamstring muscles. When in a sitting position, the ischia slide forward to draw the hamstring insertions closer together. Kyphosis worsens when the child is seated on the floor. This deformity is scored from 2 years of age.
- 0 if this deformity is absent;
- 1 if this deformity is present after 2 years.
Secondary deformities of the lower limbs Scissoring of the lower limbs
This deformity describes the crossing of the two lower limbs in extension, most often associated with an equine deformity of the foot, making standing difficult. The presence of scissoring is abnormal at any age.
- 0 if this deformity is absent;
- 2 if this deformity is present.
Permanent flexion of the hip: limitation of full extension. Permanent flexion of the knee: a limitation of full extension. Equine deformity of the foot: permanent and untreatable extension. Dislocation of the hip: a serious complication of spasticity that can be prevented by adapted positioning from birth.
- X to indicate the presence of any of these deformities.
Only the most common deformities have been mentioned here. Other abnormal standing postures should be clearly described under dystonic muscle groups and acquired muscular shortenings.
Clinical analysis of gait
The mature form of independent walking is plantigrade, beginning at 2 years: the heel touches the ground first (heel-toe). However, toe-heel gait is acceptable until 2 years. Only clinical analysis of walking (and not laboratory assessment) is included in the chart. Gait analysis requires keen observation for defining specific types of limping. The most common of these abnormalities observed in infants with cerebral palsy are listed below:
- Spastic Gait: linked to spasticity in the antigravity muscles with toe-walking and lifting of the body each time the foot touches the floor (skipping). This type of walking is only on tiptoes.
- Ataxic Gait: linked to a balance problem with broad-based stance and frequent falls.
- Hemiplegic Gait: linked to a muscular disability that makes the child move a leg in a circular pattern (scythe-like movement).
- Walking with assistance (tripod, walker, etc.) The presence of each of these abnormalities is indicated with an X (see below the functional classification for defining the effects of CP on gait).
Dystonia represents abnormal postures present at rest or hindering voluntary movement. These postures are due to co-contractions of the antagonistic muscles, caused by a lack of upper motor control. In its severe forms, dystonia causes the trunk, the neck or a part of a limb to remain fixed in an extreme position, resulting in a major functional problem. In moderate forms, it may prove difficult to distinguish from spasticity, with which it is often confused.
- 0 if dystonia is absent;
- 2 if dystonia is present.
The terms spasticity (speed-dependent) and dystonia (co-contractions due to the lack of upper motor control) are borrowed from adult neurology. In the child, this terminology is not readily applicable because of the central disorganization of the neuromotor function particular to lesions in a developing brain.
Self-regulation and signs of stress
Als, a psychologist from the Brazelton North American school of thought, proposed a conceptual framework to explain the early and progressive organization of the comportment of the newborn. She considers the newborn as being in constant interaction with the environment. The very young child’s behaviors reflect his/her level of organization and maturity and can be defined according to two poles: self-regulation and signs of stress. In an optimal situation where the stimuli from the environment are appropriate for the level of the newborn’s organization, the child is stable, showing signs of a satisfactory respiratory and hemodynamic state. He/she shows no signs of fatigue during neurological examination. Moreover, he/she seems very involved, showing a fairly calm and prolonged alertness characterized by minimal and harmonious motor activities, facial expressions of calm enjoyment, a few sucking movements with hands near face and sometimes even sucking his/her thumb. These signs of adaptation to the examination or to any other stimulus or situation are considered to be very favorable responses. Sometimes, however, in a more or less predictable manner at the beginning of examination, or if the maneuvers are inappropriate or excessive, signs of stress indicating decompensation will appear. The child looks away, his/her motor activity becomes disorganized, he/she becomes irritable or falls asleep (unable to maintain state 3). Signs of physiological instability occur, including yawning, hiccups, regurgitation, and changes in skin color, heart rate and breathing.
At term Up to 6 years A few interpretation pitfalls and recommendations Synthesis Evolutionary profiles Emerging signs during the first two years of life