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Facial electromyography in newborn and young infants with congenital facial

weakness

Article  in  Developmental Medicine & Child Neurology · July 2001

DOI: 10.1111/j.1469-8749.2001.tb00232.x · Source: PubMed

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Facial

Annotation
electromyography in
newborn and young
infants with congenital
facial weakness
Francis Renault MD, Unité de Neurophysiologie Clinique,
Hôpital d’Enfants Armand-Trousseau, 26 avenue du Docteur
Arnold-Netter, 75571 Paris 12, France.
E-mail: [email protected]

Correspondence to author at above address.

Congenital facial weakness involves various clinical manifesta- orbicularis oculi (Table I). In order to perform facial nerve con-
tions, each of which raises specific problems. The incidence of duction studies (FNCS) recording orbicularis oris responses,
congenital facial palsies ranges from 1.8 to 7.5‰ of births.1–3 stimulation is applied on the cervicofacial branch at the pretra-
Comparison of clinical and neurophysiological data enables gal point, then at a point along the horizontal portion of the
aetiologies to be differentiated, pathogenesis to be evaluated, mandible. FNCS have been performed in 211 infants without
and the prognosis to be assessed. neurological impairment between birth and 3 years.4, 5 The
facial nerve conduction velocity shows a marked increase, par-
Electrodiagnostic studies of facial muscles and nerves ticularly in the course of the first year of life during which the
NEEDLE EMG OF FACIAL MUSCLES AND FACIAL NERVE CONDUCTION average increase is 42.8% (Table II).
STUDIES
EMG activity of facial muscles is recorded by a fine concentric BLINK REFLEX
needle electrode. The skin can be anaesthetized with a lido- The blink reflex (BR) results from the contraction of the
caine/prilocaine cream. By not feeding the baby for 4 hours its orbicularis oculi muscle provoked by stimulation of a branch
cries allow bursts of activity to be recorded from the facial mus- of the trigeminal nerve.6 Stimulation of the ipsilateral nerve V
cles. EMG recording enables analysis of the recruitment pat- provokes a direct response with two components: R1 that is
tern and the average amplitude of the trace as well as the immediate and brief, and R2 that is delayed and more lasting.
morphology and duration of the motor unit potentials (MUPs; Stimulation of the contralateral nerve V provokes a crossed
see Fig. 1). The electrode is inserted in the orbicularis oris R2 response. The R1 component corresponds to an oligosy-
muscle in its external portion, more than 1 cm outside the labi- naptic reflex arc involving at least two and no more than
al commissure. The orbicularis oculi muscle is approached three synapses in the pons between the main sensory nucle-
tangentially 2 cm from the lateral angle of the eye. The us of nerve V and the motor nucleus of the ipsilateral nerve
frontalis muscle is approached tangentially above the eye- VII. The R2 component follows polysynaptic medullary path-
brow in a medial direction. The depressor anguli oris muscle ways that are slightly more caudal and closer to the bulbar
is approached 1.5 to 2 cm below the labial commissure. The formations: the spinal trigeminal nucleus and projections to
recruitment pattern of the muscles has been analysed in 32 the adjacent paramedian reticular formation and the motor
infants without neurological impairment aged from 4 days to 3 nuclei of the two nerves VII.7–10
years. Mean amplitude of the interference pattern of these In clinical practice, a single brief square wave electric shock
facial muscles is 850 µV (range 400 µV to 1.2 mV). MUP dura- (0.2ms) of low intensity (2 to 25 mA) is applied on the supraor-
tion ranges from 1 to 4.6 ms. The proportion of polyphasic bital foramen to elicit BRs that can be recorded from the orbic-
potentials is above 30%. ularis oculi muscle on both sides. The R1 response is always
A brief painless square wave electric shock (0.2 ms) of present at any age. Its latency has a very rapid maturation
supramaximal intensity (20 to 60 mA) is used for measure- achieving normal adult values between 44 weeks of concep-
ment of motor latencies. The motor response has a highly tional age and 6 months.11 The ipsilateral R2 response can be
polyphasic morphology with a mean duration from 8 to12 ms evoked in most newborn and young infants. Its latency ranges
and amplitude from 0.8 to 1.2 mV. Latencies induced by stim- from 34 to 43 ms in term newborn infants, then increases and
ulation at the pretragal point do not show significant varia- remains stable in early childhood, and decreases to adult val-
tion between birth and 3 years; latencies range from 3.6 to ues between 7 and 12 years. The contralateral R2 can be
7 ms for the orbicularis oris, and from 2.3 to 3.8 ms for the obtained in 80% of term newborn infants, but can be absent

Developmental Medicine & Child Neurology 2001, 43: 421–427 421

up to 8 months. Its latency is longer than the ipsilateral R2 ELECTRONEURONOGRAPHY
until 2 years of age. Reflex components are facilitated when Surface electrodes are placed along the nasolabial fold in
elicited after crying.11 order to record the response of the nasalis perioral muscles
elicited by a brief supramaximal electrical stimulus applied to
the facial nerve near the stylomastoid foramen during quiet
Table I: Values for motor latencies of facial muscles in wakefulness. The amplitude of the compound muscle action
children without neurological impairment. Mean (2SD) potential is recorded as a percentage of the amplitude of the
contralateral unaffected side, with an asymmetry up to 30%
Age (y) n Facial muscle (ms) being normal.12 Electroneuronography (ENOG) provides an
Orbicularis oris Orbicularis oculi estimation of the number of spared motor axons. The use of
this method in evaluating global facial nerve motor function in
0–1y 120 5.3 (1.7) 3.1 (0.8)
unilateral facial paralysis in childhood has been reviewed by
1–3y 91 5.4 (1.6) 3.3 (0.5)
Eavey and coworkers.13
Stimulation is applied at the pretragal point.
Congenital unilateral facial palsies
We have carried out a retrospective study of 172 children
with congenital facial palsy referred to our hospital for diag-
Table II: Facial nerve conduction velocity in children without nosis and treatment. The first clinical and EMG examinations
neurological impairment. Mean (2SD) were done from 3 to 90 days after birth in 103 infants, from 3
Age n Facial nerve (cervicofacial brancha), m/s
to 12 months in 49 infants, and from 1 to 2 years in the other
20 children.14
0–30d 18 19 (2.5)
1–2m 14 21.4 (3.4) PERINATAL TRAUMA TO THE FACIAL NERVE
2–4m 12 24.5 (3.9) In 85 children the congenital facial weakness was due to uni-
4–6m 24 26.3 (2.8) lateral facial paralysis without malformation (Fig. 2). Review
6–8m 23 29.1 (3.3) of this group shows that these facial palsies were of unequal
8–10m 15 31.7 (3.7)
severity and that their mechanisms were not identical. The
10–12m 14 33.3 (3.3)
12–18m 30 36.1 (3)
findings on clinical examination were not always easy to
18mo–2y 27 37.5 (4.7) interpret, especially when the paralysis was only partial. In
2–3y 34 39.5 (2.7) unilateral congenital facial paralysis the face is often quite
symmetrical at rest, and two symptoms can be helpful: the
Distance between pretragal and mandible stimulation points (mm) loss of complete closure of the eyelids and overlapping of the
are: 34.6 (2SD 6.6) up to 12 months, 48.7 (2SD 9.7) from 1 to 3 years. eyelashes, and the disappearance of the fine nostril dilation
aOrbicularis oris muscle.

Figure 1: Examples of needle EMG

a recording in orbicularis oris muscle.
(a) normal interference pattern on
crying (age 25 days; normally
developing individual). (b) full
interference pattern with brief and
polyphasic motor unit potentials
(age 12 days; congenital myotonic
dystrophy). (c) reduced interference
pattern (age 37 days; Möbius
b syndrome).

200µV

20 ms

422 Developmental Medicine & Child Neurology 2001, 43: 421–427

movements that are synchronous with breathing. Examination further strengthened by the presence of torticollis, otorrha-
while crying reveals the unilateral absence of puckering of the gia, or hemotympanum. These severe forms represented
brow, the faulty closing of the eyelids, and the asymmetry of the 46% of infants in the group delivered with forceps and 29%
mouth. The asymmetrical closing of the lips on a pacifier and in the group who were not. The immediate question is
the asymmetry of the reaction to tactile stimulation of the peri- whether surgical exploration is indicated and if so when.
oral region can also be noted in these infants. Surgery is useless if there is no curable local cause or if it is
In 48 of the 85 children with unilateral congenital facial carried out so early that it anticipates spontaneous recovery.
paralysis without malformation, extraction of the foetus On the other hand, definitive nerve lesions due to a long-
necessitated the use of forceps. Forceps delivery, birthweight standing compressive process are of major concern. Even if
above 3500 g, and primiparity are all significant risk factors no evidence of a lesion is found on CT, surgical investigation
for congenital facial palsy.3 In two children, clinical examina- should be considered if there is no sign of clinical and EMG
tion revealed a neonatal purulent otitis media. In the 35 other improvement. In our series, surgical exploration was per-
infants (35 of 85), no causal factor was found and forceps had formed in eight patients with no clinical improvement. In
not been used at birth; in such cases the possible role of pre- each of these infants two or three successive EMGs had
or perinatal compression of the extracranial part of the facial demonstrated either the persistence of a major lesion of the
nerve against the myometrium and foetal shoulder or the two branches without any sign of recovery, or partial recov-
sacral promontory should be considered, especially since ery of the cervicofacial branch but persistence of total paral-
there is often concordance between the side of facial palsy ysis of the temporofacial branch. In two infants the surgical
and that of cephalic presentation.15 EMG results enabled the investigation found that the facial nerve was compressed
hemifacial denervation to be confirmed and its severity to be against a fracture line in the petrous part of the temporal
assessed as a function of the degree and topography of axon- bone, associated in one case with chronic inflammation of
al loss as well as alterations in FNCS and BRs (Table III). In the mastoid antrum. In the six other children the facial nerve
this way, moderate forms can be distinguished in which den- was altered (pale, thin, flattened) without any obvious local
ervation is only partial and affects either the territories of the cause. Clinical and EMG monitoring of these severe forms
two branches equally or predominantly the cervicofacial showed that the process of nerve regeneration and muscular
branch. The clinical course involves progressive recovery of reinervation progresses very slowly. When the palsy seems clin-
hemifacial motor activity during the first few months and ically to remain complete, EMG may indicate a more favourable
using EMG to show the progress of nerve regeneration, prognosis by showing some voluntary MUPs and the reappear-
enabling the prediction of a favourable outcome. Moderate ance of a low-amplitude increased-latency motor response.
palsy was more frequent in the group delivered without for- ENOG may be repeated to show any progress in facial nerve
ceps (71%) compared with the group delivered with forceps function.16 Improvement in facial motor activity and EMG
(54%). In severe forms of facial paralysis, the lesion of the parameters can still be seen after the end of the second year.
temporofacial branch is total, with inactivation and no In a retrospective study of children with unilateral isolated
excitability of the orbicularis oculi muscle and abolition of congenital facial palsy who had not completely recovered by
the BRs, as well as total or partial impairment of the cervico- the age of 5 years, there was no association between this
facial branch. Such severe congenital facial palsy suggests a poor outcome and risk factors for birth trauma to the facial
fracture of the mastoid process: a diagnosis that would be nerve in 20 of 53 infants.17

Table III: Topography and severity of EMG signs of denervation

in 85 infants with perinatal stress of facial nerve

EMG results Delivery Delivery Neonatal

with without otitis
forceps forceps media
(n=48) (n=35) (n=2)

Severe palsy, n 22 10 2
TFB: total denervation, 8 3 2
BR: absent; CFB: total
denervation
TFB: total denervation, 14 7
BR: absent; CFB: partial
denervation
Moderate palsy, n 26 25
TFB: partial denervation, 12 19
BR: present; CFB: total
denervation
TFB: partial denervation, 14 6
BR: present; CFB: partial
denervation
Figure 2: Complete right facial paralysis at birth (neonatal
purulent otitis media). TFB, temporofacial branch; BR, blink reflex; CFB, cervicofacial branch

Annotation 423
ASYMMETRIC CRYING FACIES low amplitude; response to facial nerve stimulation was of
In this clinical presentation, facial asymmetry is only evident low amplitude and simplified morphology. In no case was
when the child is crying and only the lower lip is involved: there any question of total muscular agenesis. Hypoplasia of
the labial commissure does not descend on the affected side, the depressor labii inferioris muscle was also present in six
but the nasojugal folds are symmetric as is contraction of the infants. Associated congenital malformations were found in
frontalis muscle (see Fig. 3). In our series this clinical picture five of the 41 children: two cardiac septal defects, one com-
was noted in 51 of 172 infants with congenital facial paraly- plex cardiopathy, one dysmorphic syndrome, and one cleft
sis. In 10 of these 51 infants the EMG showed partial denerva- palate. The association of ACF with congenital cardiopathy has
tion of the depressor anguli oris, depressor labii inferioris, been reported under the name Cayler cardiofacial syndrome;
and mentalis muscles, leading to the diagnosis of partial the author hypothesized that an embryopathic process was
facial palsy. These 10 infants were thus included in the peri- involved.23 Bias due to recruitment in a department of paedi-
natal trauma group. This very localized topography of the atric cardiology24 or general paediatrics25 resulted in the fre-
nervous lesions corresponded to the territory of the mental quency of this association being overestimated. Prospective
branch of the facial nerve. This branch, arising from the cervi- studies of newborn infants with ACF confirmed a higher risk
cofacial branch, has a course that in the foetus and newborn of visceral malformation, particularly of the heart, great ves-
infant closely follows the base of the mandible and is thus eas- sels, and the urinary apparatus.26, 27 Deletion 22q11 has been
ily compressed.18 In fact, asymmetric crying facies (ACF) was reported with ACF as a part of the cardiofacial syndrome28
initially described as partial facial palsy.19 Electrodiagnostic and with isolated ACF.29
studies subsequently demonstrated the absence of muscle
denervation with normal values for excitability and conduc- OROFACIAL MALFORMATIONS WITH FACIAL PARALYSIS
tion of nerve VII, but revealed signs of hypoplasia of the Hemifacial malformations involving the tissue derivatives of
depressor anguli oris muscle (DAOM).20, 21 The lower part of the first and second branchial arches can indicate a lesion of
the DAOM is inserted in a diffuse manner into the platysma the V and VII cranial nerves. Facial paralysis has been reported
muscle along the base of the mandible and enters as a narrow in most infants of Goldenhar syndrome,30 in 43% of those with
fascicle into the labial commissure where its fibres merge CHARGE association (Colobomata, Heart disease, Atresia of
with those of the orbicularis oris muscle. Its contraction the choanae, Retarded growth and development, Genital
draws the labial commissure and the external part of the hypoplasia, Ear anomalies),31 and in 22% of those with hemifa-
lower lip downwards and outwards, producing an expres- cial microsomia.32 We performed bilateral facial EMG studies
sion of displeasure.22 In 41 of the 51 infants with ACF, EMG in 33 infants with asymmetric facial motility associated with
showed signs of hypoplasia of the DAOM: the interference hemifacial hypoplasia and malformation of the ear. Clinical
pattern during crying was of low-average amplitude and con- diagnoses were Goldenhar syndrome or facio-auriculo-verte-
sisted of a reduced number of MUPs of brief duration and bral spectrum,33 Treacher–Collins mandibulofacial dysostosis

Figure 3: Asymmetric crying facies: hypoplasia of depressor

anguli oris muscle. Figure 4: Facial diplegia in Möbius syndrome.

424 Developmental Medicine & Child Neurology 2001, 43: 421–427

or Franceschetti syndrome,34 and CHARGE association. EMG infants, reduced and single motor unit patterns were noted
abnormalities were either diffuse or localized to one side of in several but not all facial, lingual, and/or velopharyngeal
the face. EMG activity was either absent (10 infants), neuro- muscles; in two children all motor unit patterns showed full
genic (nine infants), or myopathic (14 infants). In this con- interference and were of low amplitude, all motor responses
text of facial malformation, we interpret myopathic EMG were of low amplitude, and the latencies and conduction
signs not as reflecting a definite myopathic process, but as an velocities were normal. In these two infants myopathy was
indication of muscular hypoplasia without denervation. In suspected, but deltoid muscle biopsy was normal and the
infants with neurogenic impairment, motor latencies were course was stable. In three children, all motor unit patterns in
increased and the FNCS showed slowed nerve conduction muscles of the face, tongue, and soft palate were typically neu-
velocity. In infants with myopathic signs, excitability thresh- rogenic; all three had experienced chronic and/or acute foetal
olds, latencies, and FNCS were normal. Among the 19 infants distress as well as severe dysphagia and respiratory complica-
with totally or partially denervated muscles, neuroradiologi- tions, and might have had hypoxic–ischaemic involvement of
cal investigation of the CNS showed a piliferous cyst of the the brain stem.
cerebellum in one case, and cerebellar hypoplasia in another.
In the 14 infants with myopathic EMG changes, no abnormal- MUSCULAR DISEASES WITH CONGENITAL FACIAL DIPLEGIA
ity of the CNS was found. Thus, for hemifacial malformations, Facial weakness is an important diagnostic indicator of con-
facial EMG and FNCS have the advantage of being able to genital myotonic muscular dystrophy,44 congenital muscular
detect whether there is involvement of the facial nerve, which dystrophy,45 and some severe congenital structural or meta-
is then a predictive sign of an associated malformation of the bolic myopathies. Facial hypotonia is associated with ptosis,
CNS, in particular of the posterior fossa.35, 36 dysphagia, and generalized paralytic hypotonia with amy-
otrophy and tendon contractures. Electrodiagnostic studies
PSEUDOPARALYSIS : ASYMMETRY OF POSTURAL ORIGIN of facial muscles and nerves demonstrate the absence of EMG
EMG can show the absence of paralysis in some congenital neurogenic signs and the normality of FNCS and BRs. EMG of
facial asymmetries noted both at rest and on crying in newborn limb muscles is necessary for demonstrating myopathic signs.
infants with no apparent malformations. Such pseudoparalysis An infantile form of facioscapulohumeral muscular dystro-
can reveal asymmetric mandibular hypoplasia, but most phy (FSH) with early facial diplegia has been described.46–48
often it is the result of intrauterine posture. A clinical feature The facial diplegia is obvious in the first year of life:49 the face
that is characteristic is the lack of parallelism of the gums. is expressionless, patients have a peculiar horizontal smile,
Pseudoparalysis was present in five of 172 infants in our con- and they may be unable to close eyes and mouth fully. In
genital facial asymmetry series. The EMG was perfectly nor- some cases, mothers noticed poor sucking at birth and lack
mal, and showed the integrity of the facial muscles and of facial expression. Sensorineural hearing loss, and tortuos-
nerves. The functional asymmetry resolved rapidly in all five ity of retinal arterioles have been observed. Motor mile-
infants within the first two months.

Congenital facial diplegia

MÖBIUS SYNDROME
Diagnosis of this syndrome is easy in cases of total or partial
facial diplegia, whether symmetrical or asymmetrical, associat-
ed with bilateral paralysis of abduction of the eye.37 When the
facial diplegia is partial, it usually involves the upper half of the
face. Abducens palsy often has the appearance of esotropia
(Fig. 4). Paralysis of the hypoglossal nerve with atrophy of
the tongue is present in one-third of cases. Most patients
have congenital dysphagia, drooling, malocclusion, velopha-
ryngeal incompetence, dysarthria, and delayed speech.38
Trigeminal nerve involvement with trismus is less frequent.
Talipes equinovarus, different malformations of hands and fin-
gers, and Poland anomaly can be associated. Most cases are
sporadic, but families with autosomal dominant inheritance
have been reported.39 The question of pathogenesis is con-
troversial as different anatomicopathological features have
been described:40 hypoplasia or atrophy of cranial nerve
nuclei, hypoplasia of the facial nerve, focal brain-stem necro-
sis, brain-stem hypoplasia, and muscular dysplasia or dystro-
phy. Most published EMG data show a predominance of
signs of axonal loss including numerous patterns showing
sparse motor units, increased latencies, slowed facial nerve
conduction velocity, and sometimes altered BRs.41 These
data support the hypothesis of a prenatal nuclear lesion of
the cranial nerves whose mechanism could be ischemic.42 Figure 5: Persistent facial weakness at 24 months of age in a
Our EMG results in a series of 22 infants were heterogeneous child with congenital dysphagia and facial diplegia (birth
but showed the predominance of signs of axonal loss.43 In 17 asphyxia in a term baby).

Annotation 425
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