CASE
REPORTIMAGING REVIEW
DIAGNOSTIC

An unusual case of bilateral anterior opercular
syndrome from a neuro-rehabilitation perspective
Chad Eventide, PA-C; Ajoy Nair, MD, FRCP; Yen F. Tai, PhD, MRCP; Katie Timms, BMEDSCI; Nicole Lichtblau, MD

CASE
A 66-year-old man was transferred to our rehabilitation
unit after treatment at another hospital for sepsis and acute
respiratory distress syndrome (ARDS).
History Four months ago, the patient presented to the
hospital with flu-like symptoms, a nonproductive cough,
shortness of breath, and confusion that had lasted 4 days.
He had stopped smoking 10 years ago after 30 years of
smoking. His medical history included hypertension,
hypercholesterolemia, ischemic heart disease, and a threevessel coronary artery bypass graft (CABG) 12 years
earlier, for which he was taking simvastatin, aspirin, and
bisoprolol.
He was admitted to the ICU because of his deteriorating
respiratory function and persistently low level of consciousness (Glasgow Coma Scale score of 11). The initial head CT,
done 4 days after the patient presented to the hospital, showed
At the Hillingdon Hospital NHS Foundation Trust in Uxbridge, United
Kingdom, Chad Eventide practices rehabilitation medicine, Ajoy Nair
is a consultant in rehabilitation medicine, Yen F. Tai is a consultant
in neurology, and Katie Timms is a speech and language therapist.
Nicole Lichtblau is a senior house officer in neuro-rehabilitation at
Charing Cross Hospital, Imperial College Healthcare NHS Trust, in
London, United Kingdom. The authors have disclosed no potential
conflicts of interest, financial or otherwise.
DOI:10.1097/01.JAA.0000554222.25758.8b
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bilateral cortical
infarcts in both
fronto-parietal
lobes, and acute
infarcts of the left
frontal lobe, right
occipital lobe, and
right cerebellum
(Figures 1 and 2). A
repeat head CT 2
weeks later showed
almost unchanged
intracranial findings. An MRI of the
brain 2 months
later showed the FIGURE 1. CT showing bilateral M4 segment
bifrontal and right (triangles) and occipital (arrow) ischemia
occipital infarcts, as
well as older right cerebellar and left caudate infarcts
(Figure 3). Electroencephalogram (EEG) showed no epileptiform activity and results could not explain the patient’s
reduced level of consciousness.
Findings of a bubble contrast echocardiogram to exclude
a patent foramen ovale, 24-hour ECG, and duplex scanning of the neck vessels were normal. The diffuse distribution of ischemic lesions in both medial cerebral artery
territories, right posterior cerebral artery territory, and
right posterior inferior cerebellar artery territory made it
likely that the strokes were embolic secondary to sepsis.
The patient was diagnosed with bilateral anterior opercular syndrome, based on the clinical syndrome of voluntary
weakness of the lingual, pharyngeal, and masticatory
muscles with preserved automatic and reflex movements
such as smiling and yawning, combined with radiologic
evidence of infarcts affecting the bilateral anterior opercula.
After 4 months of treatment in the ICU and step-down
medical unit, the patient had recovered from sepsis but
his ability to swallow and speak remained severely
impaired. He communicated by writing and some gesturing. He was transferred to our rehabilitation unit for
long-term neuro-rehabilitation.
Rehabilitation course On admission to our rehabilitation
unit, the patient was medically stable. His lipid profile and

}

ABSTRACT
Bilateral anterior opercular syndrome, also known as
Foix-Chavany-Marie syndrome, is relatively rare and is
characterized by inability of voluntary activation of facial,
lingual, pharyngeal, and masticatory muscles with preserved
automatic and reflex movements such as smiling and yawning. The syndrome is caused by bilateral lesions of the anterior opercula and results in severe impairments with speech
and swallowing. This article describes a patient with bilateral
anterior opercular syndrome secondary to embolic strokes
and how neuro-rehabilitation improved symptoms.
Keywords: bilateral anterior opercular syndrome,
Foix-Chavany-Marie syndrome, stroke, rehabilitation,
sepsis, speech

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An unusual case of bilateral anterior opercular syndrome from a neuro-rehabilitation perspective

Key points
Bilateral anterior opercular syndrome is a relatively rare
syndrome characterized by patient inability to voluntarily
activate facial, lingual, pharyngeal, and masticatory
muscles, leading to dysarthria, dysphasia, and loss of
voluntary facial expression.
Causes include stroke affecting the anterior operculum
bilateral, viral infections, ischemic lesions, tumors, and
contusion.
Ongoing rehabilitation targeted at speech, language,
swallow, dietary adjustments, and physical therapy can
help reverse the deficits over time.

inflammatory markers were within normal limits, and his
respiratory and cardiovascular examinations were unremarkable. Neurologic examination revealed weakness of
the facial and masticatory muscles innervated by cranial
nerves V and VII. The patient’s tongue, innervated by
cranial nerve XII, was hypomobile with only minimal
movement in the anterior third. Although the patient
lacked voluntary orofacial movements, he smiled involuntarily during the examination, and coughed and yawned
spontaneously throughout his admission. His mouth was
half open in resting position and his gag reflex was intact.
Cranial nerves I, II, III, IV, VI, VIII, and XI were intact
apart from saccadic horizontal eye movements and absent
convergence reaction. Sensation, strength, reflexes, gait,
and mental status were all completely normal.
The patient showed no cognitive impairments on assessment using the Oxford Cognitive Screen. This tool has
been shown effective in patients suffering from stroke and
also can be used in those with dysphagia or aphasia.1
A swallowing assessment was performed; the patient
was unable to effectively manage secretions independently
or without frequent drooling. He had moderate oropharyngeal dysphagia. The previous hospital had already
implemented a chin tuck as a strategy to facilitate airway
protection; without this posture, the patient showed evidence of audible aspiration. The patient also had severe
dysarthria combined with an element of speech apraxia,
and his phonation was rough and monotonous.
The patient was recommended for unrestricted amounts
of premashed diet and normal fluids orally; however, he
consumed only minimal amounts because he disliked the
hospital menu choices and his dysphagia prolonged meal
times. Most of his nutrition, hydration, and medication
continued to be delivered via a percutaneous endoscopic
gastrostomy (PEG) tube.
The neuro-rehabilitation program was dominated by
speech and language therapy and was focused on improving
dysphagia and dysarthria. Because of the patient’s inability
to trigger movement in the posterior two-thirds of the tongue
and reduced movement in the anterior third, speech therapy
tasks throughout admission were limited to frontal sound

productions. The
patient’s lip and jaw
movement also
were reduced, so
many productions
were indistinct and
were close approximations to sounds
or words. Therapy
tasks focused on
single-sound production, progressing
to consonant-vowel
combinations, and
then to single-word
productions. The FIGURE 2. CT showing right cerebellar
patient benefited ischemia (arrow)
from auditory and
articulatory placement cueing with visual feedback by mirror
or from the therapist during therapy sessions.
Outcome The patient was discharged from inpatient
neuro-rehabilitation after 36 days. To support further
independent practice and ongoing therapy after discharge,
the patient was provided with an app that uses videos and
sound clips to show users how to produce speech sounds
and words. After discharge, the patient also was referred
for speech and language therapy sessions in the community.
A telephone follow-up with the patient’s wife about 10
months after the initial admission to hospital confirmed
that the patient was still recovering with community neurorehabilitation support. Although his speech remained still
somewhat unintelligible, he continued to improve and had
regained his independence in daily activities.
DISCUSSION
Bilateral anterior opercular syndrome, also known as FoixChavany-Marie syndrome, is a relatively rare syndrome characterized by patient inability to voluntarily activate facial,
lingual, pharyngeal, and masticatory muscles, leading to dysarthria, dysphasia,
and loss of volunFIGURE 3. MRI showing bilateral M4
tary facial expressegment ischemia
sion; automatic and
reflex movements
such as smiling and
yawning are preserved.2 The condition is classified as a
cortical pseudobulbar palsy.
The operculum is
the part of the brain
overlying the insula.
The anterior parts
of the operculum

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27

CASE REPORT

contain the cortical areas for the motor speech center of Broca.
The difference in involuntary and voluntary movements in
patients with bilateral anterior opercular syndrome likely is
caused by a lesion in the projections from the cortex to the
nuclei of cranial nerves V, VII, IX, X, and XII.3
Causes of bilateral anterior opercular syndrome include
stroke affecting the anterior operculum bilateral, viral
infections, ischemic lesions, tumors, and contusion.4-11
The case patient’s bilateral anterior opercular syndrome
was caused by diffuse septic-embolic cerebral and cerebellar
infarcts. His presentation was somewhat unusual and included
a degree of speech apraxia and involvement of ocular movements, which may have been explained by the rather shattered
distribution of the embolic cerebral and cerebellar lesions.
The pattern of cranial nerve palsies seen in patients with
bilateral anterior opercular syndrome is believed to be
caused by interruption in the responsible motor cortex
areas, or in the projections from cortex to nuclei of cranial
nerves.9 The clinical picture of bilateral anterior opercular
syndrome also is described in patients with isolated pontine
infarct or unilateral infarcts of the anterior operculum.6,7,9,10
These symptoms may be caused by contralateral subcortical or white matter lesions—even if not visible on MRI—or
unilateral representation of the motor cortex involved in
the regulation of affected muscles in this syndrome.7,9
A differential diagnosis includes brainstem bulbar palsy
and subcortical pseudobulbar palsy, which present somewhat
similarly to bilateral anterior opercular syndrome. The main
difference is that bulbar palsy is linked to an impairment
of lower cranial nerves (IX through XII) caused by a lesion
in the brainstem or impairment of the cranial nerves outside
the brainstem. In pseudobulbar palsy, the lesion usually is
noncortical and found within the fibers connecting the
cerebral cortex with the brainstem.
Bulbar and pseudobulbar palsies have many causes, including ischemic stroke, bulbar myasthenia gravis, multiple
sclerosis, or metabolic causes. However, none show a discrepancy between voluntary and involuntary muscle activation as seen in bilateral anterior opercular syndrome. In
addition to impaired cranial nerve function, patients with
pseudobulbar palsy may have emotional lability, which is
uncommon in patients with bilateral anterior opercular
syndrome. Bulbar and noncortical pseudobulbar palsy are
associated with fasciculation of affected muscles, which is
not commonly seen in patients with bilateral anterior opercular syndrome. The prognosis is worse for patients with
bilateral lesions than for those with unilateral lesions.7 As
reported in previous case reports on bilateral anterior opercular syndrome, patient recovery from dysphagia and dysarthria is slow and in most cases incomplete.12
CONCLUSION
Because bilateral anterior opercular syndrome is rare, data
on patient prognosis and rehabilitation potential are limited. Prognosis and rehabilitation potential also depend on
28

the cause of the
bilateral opercular
lesion. In the case
patient, multiple
ischemic lesions
and septic emboli
caused bilateral
anterior opercular
syndrome. With
early identification
and intervention,
the effects may be
at least partially
reversible. Ongoing
rehabilitation targeted at speech, FIGURE 4. CT showing left anterior
language, swallow- opercular ischemia (arrow)
ing, dietary adjustments, and physical
therapy can help reverse the deficits over time. Offer patients
interventions for secondary prevention of stroke to prevent
further brain damage. JAAPA
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