Foix–Chavany–

Marie syndrome

Foix-Chavany-Marie Syndrome (FCMS),

also known as Bilateral Opercular
Syndrome, is a neuropathological disorder
characterized by paralysis of the facial,
tongue, pharynx, and masticatory muscles
of the mouth that aid in chewing.[1] The
disorder is primarily caused by thrombotic
and embolic strokes, which cause a
deficiency of oxygen in the brain. As a
result, bilateral lesions may form in the
junctions between the frontal lobe and
temporal lobe, the parietal lobe and
cortical lobe, or the subcortical region of
the brain.[2][3] FCMS may also arise from
defects existing at birth that may be
inherited or nonhereditary. Symptoms of
FCMS can be present in a person of any
age and it is diagnosed using automatic-
voluntary dissociation assessment,
psycholinguistic testing,
neuropsychological testing, and brain
scanning. Treatment for FCMS depends on
the onset, as well as on the severity of
symptoms, and it involves a
multidisciplinary approach.
 Foix–Chavany–Marie syndrome
Other names Facio-pharyngo-
glosso-masticatory
diplegia

Operculum (brain)

Specialty Neurology

Classification
Frontal (coronal) section human brain. Lesions
occurring in the highlighted regions are characteristic
of Foix-Chavany-Marie Syndrome.

There are two forms (also referred to as

"classifications") of FCMS; bilateral and
unilateral. The bilateral form is most
common (also referred to as the "classical
form") and is caused by the formation of
lesions on both sides of the anterior or
posterior region of the operculum. In
contrast, the unilateral form is rare and is
caused by the formation of lesions on one
side of the anterior or posterior region of
the operculum.[1] Lesions located in the
anterior regions of the operculum are
associated with motor deficits and
anarthria, a total absence of the ability to
form speech or language. Lesions located
in the posterior regions of the operculum
are associated with parietal opercular
functions.[2] The two classifications of
FCMS were established based on the
location of the lesion, stroke, and trauma
affecting the brain. Classifying FCMS
based solely upon lesions yields five
specific subtypes of FCMS currently
known to fall into the bilateral and
unilateral categories:[1][3]

Bilateral anterior opercular syndrome

(lesion in both the anterior or in the
frontal operculum)
Opercular-subopercular syndrome
(lesions in the opercular cortex on one
side and the subopercular lesion in the
contralateral side).
Subopercular syndrome (lesions in the
subcortical corticobulbar projections
only).
Unilateral anterior syndrome involving
the frontal operculum.
Posterior syndrome involving the
junction between the frontal and the
parietal lobe of the operculum.
Bilateral …

The bilateral form of FCMS (also known as

facio-labio-pharyngo-glosso-laryngo-
brachial paralysis) is consistent with the
classic presentation of bilateral
corticobulbar involvement. It is
characterized by well-preserved automatic
and reflex movements. It is caused by
lesions in the cortical or subcortical region
of the anterior opercular area surrounding
the insula forming the gyri of the frontal,
temporal, and parietal lobes.[4]
Unilateral …

The unilateral operculum syndrome is a

very rare form of FCMS caused by the
formation of unilateral lesions. In this form
of FCMS, the unaffected hemisphere of
the brain compensates for the unilateral
lesion. Usually, this occurs when the
unaffected region is the individual's
dominant hemisphere.[1]

Symptoms
An individual affected with FCMS develops
disabilities associated with voluntary
movements using the facial, lingual,
pharyngeal, and masticatory muscles.
However, the reflexive and autonomic
functions of these muscles groups are
usually intact. Common symptoms include
drooling, an inability to elevate and
depress the mandible, difficulty chewing,
inability of protruding tongue, swallowing,
and loss of speech.[3][1][4]
Classification of the disorder is
distinguished by the location of the lesions
formed, which causes certain symptoms
to be present or amplified. FCMS caused
by the formation of bilateral lesions
causes paralysis of the facial, lingual,
pharyngeal, and masticatory muscles. This
form of FCMS involves voluntary-
autonomic dissociation and an inability to
form speech. The formation of bilateral
lesions confined to the posterior
operculum has a distinct symptom of word
deafness, an inability to understand
language.

FCMS caused by the formation of lesions

unilaterally causes muteness of speech
and upper motor neuron cranial nerve
paresis, muscular weakness. The
formation of unilateral lesions confined to
the posterior operculum has distinct
symptoms that includes sensory loss in
the hand and face contralateral to the
location of the lesion.[4]
Causes
Foix-Chavany-Marie Syndrome is primarily
caused by multiple strokes and lesions.
However, less common causes that can
eventually produce lesions to the
operculum resulting in the FCMS
syndrome include the following; tumors,
trauma, encephalitis, neurodegenerative
diseases, and vasculitis. Viral infections,
such as Herpes and HIV can also cause
FCMS. Moreover, any lesion in the cortical
or sub-cortical region affecting the
corticobulbar pathways will produce
FCMS.[1]

Cerebrovascular disease …

Strokes are one of the most common

causes of Foix-Chavany-Marie Syndrome.
The type of strokes associated with this
syndrome include embolic and thrombotic
strokes. Strokes affecting the middle
cerebral artery and the branches that pass
through or near the operculum are
characteristic of FCMS.[1]

Central nervous system infection …

Symptoms of infections specifically HIV

and Herpes simplex encephalitis can
cause FCMS. Numerous lesions can
develop with HIV infections, which likely
result in the development of FCMS.[1][5]

Epilepsy …
Epilepsy symptoms such as seizures can
spread discharges that cause FCMS. This
causation results in the only reversible
development of FCMS as it is the only
cause that allows full recuperation from
speech, swallowing, and mastication
difficulties when treated.[3] This causation
is most commonly seen in children with
FCMS.[3]

Unusual Causes …

Tumors
 Multiple Sclerosis
Neurodegenerative diseases
Acute disseminated encephalomyelitis
Moyamoya disease
Vasculitis[6]
Trauma

Mechanism
FCMS is primarily originates from
damages in the posterior region of the
inferior frontal gyrus and inferior region of
the precentral gyrus.[6] Anatomically, the
word operculum is defined as the cortices
encompassing the insula, which includes
the pre and post-central, inferior-frontal,
supramarginal, angular inferior parietal,
and superior temporal convolutions.[1]
Parts of the brain such as Heschl's gyrus,
Broadmann's area, Broca's Area,
Wernicke's Area are amongst the most
relevant in the operculum. These areas are
responsible for auditory functions for
language and speech.[7]
Operculum of the inferior frontal gyrus.

FCMS, sometimes called cortical

pseudobulbar palsy, is characterized by
lesions affected both sides of the
operculum. These lesions typically
damage the cranial nerves leading to both
motor and sensory deficits. The cranial
nerves that are impaired include the
following; Cranial Nerve (CN) V (the
trigeminal nerve), CN VII (the facial nerve),
CN IX (the glossopharyngeal nerve), CN X
(the vagus nerve), and CN XII (the
hypoglossal nerve). Cerebral
malformation, namely unilateral
schizencephaly in association with
contralateral polymicrogyria symmetrically
in the perisylvian area is another known
charactheristic of FCMS. Moreover,
another deformation found with FCMS
includes the failure of opercularization
caused by the underdevelopment of the
anterior part of the opercula found in the
fetal brain in the 32nd week.[7]

Neuropathology …

The anatomic basis for the automatic

voluntary dissociation is characterized by
the following. Neurons that lie adjacently
in the operculum project supranuclear
fibers to the cranial nuclei for the voluntary
movement of facial, phanryngeal, lingual,
and masticatory muscles. Emotional
movement of these muscles is controlled
by alternative pathways that run from the
amygdala and lateral hypothalamus to the
brainstem via the medial forebrain bundle
and dorsal longitudinal fasciculus.[8]

The opercular cortex surrounding the

insula is separated by two anatomical
components: the ascending rami of the
lateral sulcus and the posterior rami into
three different sections of the
operculum.[3]
 1. Frontal operculum formed by
posterior part of the inferior frontal
gyrus.
2. Fronto-parietal opercula formed by
the lowermost part of the precentral
and postcentral gyrus and the
anterior and lowermost part of the
inferior parietal lobule.
3. Temporal opercula formed by the
superior temporal gyrus.[3]

Diagnosis
Criteria …

FCMS shares similar characteristics with

the following disorders: catatonia, akinetic
mutism, orbuccal apraxia, Broca’s aphasia,
pseudobulbar palsy, bulbar palsy
secondary to myasthenia gravis, Guillain-
Barré syndrome, and brainstem strokes.[1]

In determining a diagnosis between with

catatonia, akinetic mutism, and FCMS, a
person must demonstrate their ability to
perform voluntary function of the limbs.
Patients with catatonia or akinetic mutism
are not able to perform voluntary
commands that involve the use of limbs,
while patients with FCMS still possess
voluntary usage of limbs.[1] If a person can
demonstrate ability in voluntary usage of
limbs, catatonia and akinetic mutism are
most likely ruled out from the diagnosis.

In determining a diagnosis between

Broca’s aphasia and FCMS, a person must
demonstrate their ability in voluntary
movement of cranial musculature. People
with Broca’s aphasia may not exhibit a
complete loss of voluntary movement
facial muscles, pharyngeal muscles,
laryngeal muscles, brachial muscles,
tongue muscles, and muscles of the
mouth that aid in chewing. These
voluntary functions may still be present, to
varying degrees. People with FCMS do not
possess this ability. For people with FCMS,
voluntary movement of cranial
musculature is completely absent.[1]
In determining a diagnosis between
pseudobulbar palsy, a person must
demonstrate whether or not muteness is
present, as well as the ability to move the
facial, buccal, lingual, and pharyngeal
muscles. People with pseudobulbar palsy
exhibit, to varying degrees, an ability in
these functions, while patients with FCMS
do not.[1]

Techniques …
There are three general classes of tests
utilized by physicians when determining a
diagnosis for FCMS: (1) automatic-
voluntary dissociation assessment, (2)
psycholinguistic testing, and (3)
neuropsychological testing.[9] In addition,
brain scanning techniques are utilized to
observe whether ischemic abnormalities
or lesions are present within the
operculum region of the cortices.

Automatic-voluntary dissociation
assessment …
FCMS is largely characterized by the
paralysis of voluntary movement in facial,
lingual, pharyngeal, and masticatory
muscles, while automatic, involuntary
functions of these four muscle groups
remain.[3] Automatic functions are
performed by inducing involuntary
reflexes, such as palatal, laryngeal, blink,
and gag reflexes. Other involuntary
functions that are tested include
spontaneous smiling, laughter, and
yawning.[10] Patients with the disorder are
able to these functions under automatic,
involuntary reflex. An individual’s ability to
perform these functions voluntarily are
tested determined through a series of
commands by the physician. Typically,
individuals with the disorder are not able
to perform any of these functions upon
command. Dissociation between
automatic and voluntary dissociation is
indicated by an individuals’ ability to
perform the involuntary, automatic
functions, and their inability to perform the
same actions, voluntary.

Psycholinguistic testing …

Psycholinguistics pertain to the

psychological and neurobiological
components that allow humans to acquire,
utilize, comprehend, and produce
language. The tests most commonly used
for psycholinguistic testing include the
Dutch version of Aachen aphasia test,
syntactic comprehension test, and the
Token test.[10] Psycholinguistics allow
physicians to narrow down and rule out
other disorders that may be similar to
FCMS when diagnosing a patient.

Neuropsychological testing …

Neuropsychology is the study of

neurobiology and psychology.
Neuropsychological tests are utilized for
the purpose of observing an individuals’
abilities in cognitive functioning,
reasoning, and memories.[10] The tests
most commonly used for
neuropsychological testing include WAIS-
III, Stroop test, Bourdon Wiersma test, and
the Rey-Osterrieth complex figure test.
These tests allow physicians to evaluate
the degree to which the bilateral lesions in
the operculum have been affected, and
allow for the determination of proper
treatment.

Imaging …
Scanning techniques include EEG, SPECT,
MRI, and CT brain scanning.[1][2] These
additional techniques are useful in
determining what type of lesion the patient
has, and allows physicians to determine
more effective ways in treating the patient.

(A) CT scan of a patient with middle cerebral artery

stroke illustrating hypodensity areas within the
temporal and frontal lobes. (B) CT scan displaying an
i h i t k b d i th f t i t l
ischemic stroke bordering the fronto-parietal
opercular cortex (red arrow) and a left-sided ichemic
lesion of the fronto-parietal opercular cortex (blue
arrow).

Magnetic resonance imaging …

MRI is one of the best techniques that can

detect the lesions in the brain of the FCMS
that some of the times are missed by just
using a Computer-Tomography Scan. Also,
this type of imaging can reveal right frontal
lobes contusions encompassing the
anterior operculum, the premotor area, and
the association area.[10]

CT Scan …

This computer-tomography type of

imaging is one of the most used in any
clinical environment and although it can
detect some of the brain areas affected by
a stroke or a trauma it does not provide
the same acuity as the magnetic
resonance imaging. CT scans can also
reveal, in patients with the syndrome, the
bilateral cortical infarcts located in the
posterior frontal region involving the
opercular areas.[3]

SPECT …

The single photon emission computed

tomography of the brain can show uptake
area in the right frontal lobe and normal
uptake are in the left hemisphere.[10] This
type of imaging can give a more detailed
view of a specific region of the brain.
Other techniques …

An electroencephalography (EEG) is also

used in patients with the FCMS and it can
reveal focal slowing and epileptic
discharges from left fronto-temporal
regions.[3]

Management
Treatment of Foix–Chavany–Marie
syndrome depends on the onset of
symptoms and involves a multidisciplinary
approach. Drugs are used in neurological
recovery depending on the etiological
classification of FCMS. FCMS caused by
epilepsy, specifically resulting in the
development of lesions in the bilateral and
subcortical regions of the brain can be
treated using antiepileptic drugs to reverse
abnormal EEG changes and induce
complete neurological recovery.[3] In
addition, a hemispherectomy can be
performed to reverse neurological deficits
and control the seizures. This procedure
can result in a complete recovery from
epileptic seizures.[3] Physical therapy is
also used to manage symptoms and
improve quality of life. Classical FCMS
resulting in the decline of ones ability to
speak and swallow can be treated using
neuromuscular electrical stimulation and
traditional dysphagia therapy. Speech
therapy further targeting dysphagia can
strengthen oral musculature using
modified feeding techniques and postures.
Therapeutic feedings include practicing
oral and lingual movements using ice
chips.[1] In addition, different procedures
can be performed by a neurosurgeon to
alleviate some symptoms.

See also
Pseudobulbar Palsy
Operculum
Corticobulbar Tracts
Wernicke's Aphasia
Broca's Aphasia
References
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"The opercular-subopercular
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2. Starkstein, SE; Berthier, M; Leiguarda,
R (July 1988). "Bilateral opercular
syndrome and crossed aphemia due
to a right insular lesion: a
 clinicopahological study". Brain and
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Rana (2010). "Opercular syndrome:
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(PDF). Neurology Asia. 15 (2): 145–
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4. Desai, SD; Patel, D; Bharani, S; Kharod,
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6. Milanlioglu, A; Aydın, MN; Gökgül, A;
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 9. Theys, Tom; Van Cauter, Sofie; Kho,
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Further reading
Ole Daniel Enersen. "Foix-Chavany-Marie
syndrome" . Who Named It?. Retrieved
2006-07-25.

External links
Classification ICD-10: G12.2 • D

MeSH: C537069

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