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 ante- rior
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.
Le syndrome bi-operculaire, également
dénommé syndrome de
Foix-Chavany-Marie, est relativement rare et se
caractérise par une incapacité
d'activation volontaire des muscles du visage,
de la langue, du pharynx et de la mastication,
alors que les mouvements automatiques et
réflexes sont préservés,
sourire et bâillement
notamment.
Le syndrome de Foix-Chavany-Marie (syndrome
de FCM) est une paralysie suprabulbaire ou
pseudobulbaire cortico-souscorticale des nerfs
crâniens VII, IX, XI, XII
caractérisée par une dysarthrie
sévère et une dysphagie
associée à une paralysie
facio-linguo-masticatrice centrale
bilatérale, avec une importante
dissociation automatico-volontaire et
préservation des mouvements involontaires
des muscles touchés.
Cet article décrit un patient atteint
du syndrome bi-operculaire antérieur
secondaire à un AVC embolique et la
façon dont la neuro
rééducation a
amélioré ses symptômes
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 three- vessel 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 bilateral cortical infarcts in
both fronto-parietal lobes, and acute infarcts
of the left frontal lobe, right occipital lobe,
and right cerebellum. A repeat head CT 2 weeks
later showed almost unchanged intracranial
findings. An MRI of the brain 2 months later
showed the bifrontal and right occipital
infarcts, as well as older right cerebellar and
left caudate infarcts. 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 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 pro-
duction, progressing to consonant-vowel
combinations, and then to single-word
productions. The patient benefited 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 neuro-
rehabilitation 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 Foix- Chavany-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 voluntary facial expression; automatic
and segment ischemia reflex movements such as
smiling and yawning are preserved. 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 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.
Causes of bilateral anterior opercular
syndrome include stroke affecting the anterior
operculum bilateral, viral infections, ischemic
lesions, tumors, and contusion. 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. The clinical picture
of bilateral anterior opercular syndrome also is
described in patients with isolated pontine
infarct or unilateral infarcts of the anterior
operculum. These symptoms may be caused by
contralateral subcortical or white matter
lesions&emdash;even if not visible on
MRI&emdash;or unilateral representation of the
motor cortex involved in the regulation of
affected muscles in this syndrome.
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.
CONCLUSION
Because bilateral anterior opercular
syndrome is rare, data on patient prognosis and
rehabilitation potential are limited. Prognosis
and rehabilitation also depend on 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, language, swallowing,
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.
REFERENCES
1. Demeyere N, Riddoch MJ, Slavkova ED, et
al. The Oxford Cognitive Screen (OCS):
validation of a stroke-specific short cognitive
screening tool. Psychol Assess.
2015;27(3):883-894.
2. Weller M. Anterior opercular cortex
lesions cause dissociated lower cranial nerve
palsies and anarthria but no aphasia: Foix-
Chavany-Marie syndrome and "automatic voluntary
dissocia- tion" revisited. J Neurol.
1993;240(4):199-208.
3. Christen HJ, Hanefeld F, Kruse E, et al.
Foix-Chavany-Marie (anterior operculum) syndrome
in childhood: a reappraisal of Worster-Drought
syndrome. Dev Med Child Neurol.
2000;42(2):122-132.
4. Matsushima T, Nishioka K, Tanaka R, et
al. Anterior opercular syndrome induced by
Epstein-Barr virus encephalitis. Neurocase.
2016;22(1):103-108.
5. Conforti R, Capasso R, Capaldo G, et al.
Foix-Chavany-Marie syndrome in a 17-year-old
female with congenital cytomegalovi- rus
infection. Neuropsychiatr Dis Treat.
2014;10:2249-2252.
6. Kozak HH, Uca AU, Du_ndar MA.
Foix-Chavany-Marie syndrome after an isolated
pontine infarct: a 7-year follow-up. Neurol
India. 2015;63(6):983-985.
7. Sa_ F, Menezes Cordeiro I, Mestre S,
Nzwalo H. Unilateral opercular infarction
presenting with Foix-Chavany-Marie syndrome. BMJ
Case Rep. 2014;2014:bcr2014206439.
8. Ohtomo R, Iwata A, Tsuji S. Unilateral
opercular infarction presenting with
Foix-Chavany-Marie syndrome. J Stroke Cere-
brovasc Dis. 2014;23(1):179-181.
9. Bradley N, Hannon N, Lebus C, et al.
Bilateral corona radiata infarcts: a new
topographic location of Foix-Chavany-Marie
syndrome. Int J Stroke. 2014;9(8):E39.
10. Popescu M, Sandu AM, Onose G, et al.
Foix-Chavany-Marie syndrome caused by bilateral
opercular lesions: right side tumor and left
side ischemic stroke. Rev Neurol.
2013;57(7):333-335.
11. Nitta N, Shiino A, Sakaue Y, Nozaki K.
Foix-Chavany-Marie syndrome after unilateral
anterior opercular contusion: a case report.
Clin Neurol Neurosurg.
2013;115(8):1539-1541.
12. Milanlioglu A, Aydõn MN, Go_kgu_l A, et
al. Ischemic bilateral opercular syndrome. Case
Rep Med. 2013;2013:513572.
