f) Neurological assessment (using axial tone) at 40 weeks of gestational age

(Dr. Amiel Tison1i)

During the early critical phase, it is not possible to clinically assess neurological
function in very preterm infants, since they are very small; they may be unstable
and / or ventilated.
This neglect to collect clinical information is understandable in the acute phase of
neonatal adaptation, but seems unjustified when the child recovers, can breathe on
his own and is able to tolerate some manipulation. At this point, and especially
when the preterm baby reaches 40 weeks, the neurological evaluation can offer
objective data on the anomalies or confirm normality.

A normal neurological evaluation at 40 weeks corrected age is sign of a favorable

outcome, if the results from the cerebral ultrasound are normal.

1Clinical assessment of axial tone

Definition of muscle tone

- Passive tone is observed one segment at the time, with maneuvers that evaluate
the amplitude of a slow stretching executed by the observer, the child is passive;
the result is systematically measured or graded and expressed as an angle or as
the amplitude of displacement.
-Active tone is the muscular activity of the infant, as he responds to the examiner’s
maneuvers; the results are expressed by the presence or existence or absence of
an active motor response.

2. Normal findings in full-term infants

Posture
At rest, in supine position, the neck muscles are relaxed and there is very little or
no space between the examination couch and the spine.

Repeated ventral flexion of the head

On repeated flexion of the head, there should be no increase in resistance at the
third or fourth trial.

Passive tone in the trunk

Ventral flexion: with the child in supine, the examiner grasps the lower limbs and
pushes legs and pelvis towards the head, looking for maximum curvature of the
spine. Some passive flexion of the trunk is normally present.

The Newborn Infant: One Brain for Life by doctors Claudine Amiel-Tison and Ann
1

Stewart (Les Éditions INSERM) by permission of Dr. C. Amiel-Tison.

Dorsal flexion: while the infant is laying on his/her side, the examiner places the
palm of the hand on the infant’s lumbar region, while pulling both legs backwards
with the other. There is minimal or no extension.

Comparison between flexion and extension: there is great individual variability in

the amplitude of flexion and extension in all ages, but in the normal individual,
flexion always surpasses extension.

Active tone in the neck

“Pull-to-sitting” maneuvers and reverse “sitting-to-lying” are used to separately

assess the reactions of the flexor and extensor muscles. Neck extension and
flexion and the balance between the two are evaluated by eliciting alternate
contraction of the neck flexor and extensor muscles, by gently rocking the child’s
trunk about the vertical. From the first days of life of a normal full-term newborn,
these movements are symmetrical and are described as “symmetrical breaking”.
Performing this maneuver requires practice so that an appropriate speed to elicit
these signs from the maneuver: if too brisk, the passive movement of the head
occurs; if too slow, the absence of a response may be misinterpreted.

Active tone in the trunk and lower limbs

Upright position: a) it is normal when the child is held vertically, he or she leans
slightly forward, sustaining his or her own weight; b) when there is global
hypertension of trunk and lower limbs, the body is arched.
Maturation of axial tone

The maturation process of the tone of extensor muscles is more precocious than
that of the flexor muscles. A perfect balance is only obtained around full term (40
weeks of corrected age), and a complete control of the head at six to eight weeks.
Once this control is achieved, we assume that control of cortical structures has
supplanted that of sub cortical ones.

3. Abnormal findings

Active and passive tone may be abnormal in several different ways. Specific types
of abnormalities can be identified and they point to underlying pathology.

Posture

Supine position

A: retrocolis: posture with hyperextension of the head only

B: opisthotonos: posture with backward arching of neck and trunk

Repeated ventral flexion of the neck

When repeated passive flexion of the neck elicits an increased resistance, it shows
hypertonicity of the extensor muscles of the neck.
Passive tone in the trunk

Ventral flexion and dorsal extension: a-d normal balance, more flexion than
extension; b-e or b-f: abnormal balance with moderate or excessive extension, and
no flexion; c-f: global hypotonia with unlimited flexion and extension; b-d: global
stiffness, from a tendency in the family or poor cooperation from the child.

Active tone of the neck, abnormal responses

Global hypotonia: a. the passage of the head both forward and backward is
passive and there is no active response whatsoever; b. hypotonia confined to the
flexors: passive passage forward, active passage backward; c. hypertonia in the
extensors: passage forward is difficult but active, passage backward is “too good”
(NB: typical “chin forward position” as child leans forward).

Active tone in the trunk and lower limbs

Weak or absent global straightening is evident by attempting to place the child in a

standing position: no straightening occurs and no contraction is observed in the
spinal muscles. Excessive tone is indicated by arching the back, many times
associated with pointed feet and crossed, scissor-like legs.

4. Clustering of signs: description of three syndromes

Global hypotonia

Active and passive tone of flexor and extensor muscles of the axis is almost
absent. In the newborn, these signs are frequently associated with hypotonia of the
limb muscles. This easily recognized condition is usually referred to as “rag doll”.

Severe, generalized hypotonia is a frequent finding during the acute period of any
brain lesion in the neonatal period; it is non-specific. For instance, it can be
observed during the first stage of a hypoxic-ischemic injury, following to ICH or
hyper bilirubinemic encephalopathy. In these cases, hypotonia is of a central origin
and just a manifestation of a severe CNS depression. Systemic infection and
sedative medication administered to the infant or the mother can produce a similar
clinical picture, although the mechanism causing the hypotonia is different. Axial
hypotonia can also be the main sign of spinal or muscular disorders in the neonatal
period. Clinical diagnosis is made by revising the gestational history (diminished
fetal movement, polyhydramnios) and the clinical features of the newborn: the
quality of alertness, cry, eye movement, deep tendon reflex, muscular bulk, tongue
fasciculation and topography of motor deficit. However, in order to reach a
diagnosis, additional laboratory investigation is required.

Hypotonia confined to the axial flexor muscles

A combination of symptoms, presented in the table below, comes from the inability
of weak or deficient flexor muscles (cortical control) to balance the normal extensor
tone (under sub-cortical control). In severe cases, this condition is easily
recognized as the opisthotonic posture. In contrast, in less severe cases, there is
nothing remarkable about the posture, and the condition can be identified by the
response to the “pull to sit” maneuver, showing imbalance between an poor
response from the ventral flexors and a good dorsal extension with a normal
resting position of the head on the chest, at the end of the maneuver. Often,
hypotonia of the shoulder girdle (scarf) is associated.
Hypotonia of the axial flexors muscles is nearly a constant in moderate or mild
grades of hypoxic-ischemic encephalopathy in the full-term newborn, or in the
recovery phase of the severe grade. It is not an isolated finding but rather one of
several that comprise a clinical picture that defines the hypoxic-ischemic
encephalopathy.

Clinical findingsin hypotoniaconfined to axial extensor muscles

Posture Normal or mild to severe opisthotonos

Repeated flexion of the head No increase in resistance
Passive tone of trunk More extension than flexion
Active tone
-Neck flexor muscles - Passive passage forward (no visible
contraction)
- Normal resting posture on the chest
at the end of the raise to sit.
- Neck extensor muscles Normal, active
- Straightening reaction Normal or excessive with arching
Associated finding in upper limb Decrease of passive and active tone
girdle (scarf, recoil and response (large scarf, no recoil poor or no
to traction). response to traction.

Hypertonia of the axial extensor muscles

The combination of signs presented in the table below result from increased axial
extensor tone in the presence of normal flexor tone. During the “raise to sit”
maneuver, the head does not move forward; the maneuver ends with the “chin
poking forward” position due to excessive extensor tone. Hypertonia of the
trapezius muscle, inserted in the scapula, results in external rotation of the
shoulder, “high” elbows a “tight” scarf and a permanent “candlestick” posture.
There may be a number of underlying causes for these symptoms; therefore
interpretation must be done within the clinical context of each case.

The candlestick posture is the result of the permanent contraction (shortening) of

the trapezius muscle (NB: the deep fold on the posterior aspect of the arm is the
consequence of permanent external rotation).

Clinical findings in hypertonia of the axial extensor muscles

 Posture Mild to severe opisthotonos
Repeated flexion of head Increase of resistance
Passive tone of trunk More extension than flexion
Active tone

- Neck flexor muscles -Difficult passage forward (with

visible contraction)
-“Chin forward” position at the
end of raise to sit
- Neck extensor muscles “Too good passage backward
- Straightening reaction Excessive with arching
Associated findings in upper limb Markedly abnormal passive tone
girdle (“candlestick” posture)

Raised intracranial tension

A combination of neurological and cranial signs helps identify this condition. The
magnitude of cranial signs depends on the maturation status of the skull bones.
Permanent or intermittent “sunsetting” may occur in addition to the predominance
in the tone of the extensors. Other neurological signs, such as yawning,
drowsiness, lethargy, irregular breathing, apneic episodes, bradycardia and
vomiting may be associated.

Physiopathology of axial extensor hypertonia in the presence of intracranial

hypertensionis speculative. Observation of premature newborns with intracranial
hypertension, after excessive sodium and water intake, shows that the extensor
hypertonicity is mechanical and completely reversible after a lumbar puncture. It is
generally accepted that in adults, extension of the neck is a reflex mechanism
triggered by pain from meningeal irritation and traction of the spinal nerve. Instead,
since flexion of the head increases the CSF tension in the cisterna magna, reflex
extension rigidity may alleviate it. Compression or irritation of the spinal branch of
the accessory nerve, at foramen magnum level may explain the contraction of the
trapezius muscle. While purely mechanical, this mechanism could decrease
pressure and alleviate pain.

Meningitis

In nearly all cases, diagnosis is made before the appearance of evident

neurological signs. The mechanism that produces hypertonia in axial extensor
muscles in meningitis is the same one responsible for raised intracranial tension,
which is one of the first symptoms of meningeal inflammation.

Bilirubinencephalopathy
In the past, opisthotonos has been described in the second phase of kernicterus.
Since brain lesions in this pathology are mostly found in the basal ganglia and
brain stem nucleus, there may not be cortical involvement. Today, this pathology is
very rare and is commonly associated with an anoxic cerebral lesion.

Gastro-esophageal reflux (GER)

Opisthotonos has been described as a sign of GER, appearing to mimic

neurological or psychiatric disorders. In GER, opisthotonos appears in episodes of
30 to 60 seconds, a probable response aimed at avoiding painful esophagitis at the
often resulting from GER. Other signs, such sudden stare, changes in skin color
and apnea. Esophageal spasm episodes have been documented in infants,
associated with an anxious stare followed by agitation, hyper-extension of neck
and back, apnea and bradycardia.

Avoidance behavior: a possibly psychogenetic etiology of opisthotonos

In 1962, Ajuriaguerra described the “le dialogue tonique mère-enfant”, suggesting

that modifications in muscle tone are used as mediators of the mother-child
interaction. When there are difficulties in that relationship, there may be changes in
tone which simulate a neurological pathology. This may appear after the neonatal
period, usually at three months or later.

Short trapezius muscle: a diagnostic hazard

The maneuvers described above for evaluating active tone of the neck muscles
assume that the muscle length is normal. Nevertheless, impairment of one muscle
set may appear as an abnormal response of central origin. Different pre and post
natal situations may cause individual or groups of muscles to shorten, hindering
normal responses and making the interpretation of the maneuvers difficult.

Prenatally

An asymmetrical posture of head and neck, in utero, with rotation and flexion to
one side, will cause the shortening of one of the sternocleidomastoid muscle, while
the counter lateral muscle will elongate.

A head in hyper-extension, following a breech presentation, also produces

abnormal posture of head and neck. The group of extensor muscles is shortened
and the flexor group is elongated. Because of this, the flexor response to the “pull
to sit” maneuver is poor and the head does not deop forward at the end of the
maneuver.

Post-natally

A shortening of the trapezius muscle may be produced if the infant is nursed in the
prone position for long periods of time, with the head extended to facilitate
mechanical ventilation. This situation is frequent in premature infants. In mature
infants, this condition is caused by the use of some types of baby carriers, which
do not maintain the head in the axis.

The clinical features of the short trapezius muscle are summarized in the table
below. These are similar to those of hypertonia of the neck extensor muscles. For
a differential diagnosis, Grenier offers different clinical clues to help distinguish a
short trapezium from opisthotonos of central origin.
- The trapezius’ tendon remains very tense, even if the child is awake and quiet,
and this is not modified with attempts to relax the baby.

- The trapezius muscle is hypertrophic; its triangular aspect can be easily seen
from under the skin.

- There is no increase in resistance with repeated flexion of the neck.

Clinical finding in short trapezius

Posture Minor to severe opisthotonos

very tense muscle even after
relaxation
often hypertrophic trapezoid muscle

Repeated head flexion No increase in resistance (short

muscle, but normal elongation
reflex)
Passive trunk tone More amplitude in extension than in
flexion
Active tone
- Difficult passage forward (with
- Neck flexor muscles visible muscle contraction)
- “Chin forward” position at the end
of the raise to sit

“Too good” passage backwards

- Neck extensor muscles
Excessive with arching

- Straightening reaction
Associated findings in upper limb Markedly abnormal passive tone
girdle (candlestick posture)

Risk circumstances Prenatal deformation

Protracted mechanical ventilation
Predominant prone posture
Inadequate baby carrier
Additionally, authors such as Grenier and Towen emphasize that short trapezius
are frequently an isolated anomaly, without involving other abnormal CNS signs.
Physical therapy rapidly has a beneficial effect on the shortening of the trapezius
muscle and helps differentiate findings with central and peripheral origin in difficult
cases. Not recognizing the origin of an abnormal posture may lead to an
excessively bleak prognosis. In order to facilitate the neurological evaluation, to
prevent the shortening of the muscle, and to avoid such an error, preventive
physical therapy should be introducedas soon as an abnormal posture is observed,
regardless of its origin.

The literature generally agrees that changes of the axial tone are important. By
paying attention to these signs, using a standardized clinical method it is possible
to follow the evolution of a brain lesion from the neonatal period through childhood,
and maybe understand the relationship between early injury and later childhood
disabilities. This is a sure way to learn to recognize children who will benefit from
an active intervention at the earliest possible time.

To conclude, in spite great progress in the detection and diagnosis of brain

damage in the newborn, through morphological investigation and
electrophysiological techniques, the contribution of the clinical examination will
always be indispensable. Neurological signs, especially those observed on the
axis, provide important clues to the pathology of perinatal brain damage. They
also facilitate following the evolution of brain damage during infancy and
childhood. In numerous cases of moderate damage or dysfunction, the signs
observed in the neonatal period are the only evidence to establish a
relationship between early events to late neurological or psychological
outcome. Ideally, these signs must be sought during the neonatal period,
following any abnormalities into infancy. Even if these signs are only found in
school-age children being examined for the first time, they offer valuable
evidence of the child’s problem early origin.
i