Rare case of bilateral carotid artery dissection
presenting with Foix-­Chavany-­Marie syndrome
Rajveer Singh ‍ ‍, Alex Rebello ‍ ‍, Nandita Prabhat, Dheeraj Khurana
Neurology, Post Graduate
Institute of Medical Education
and Research, Chandigarh, India
Correspondence to
Professor Dheeraj Khurana;
​dherajk@​yahoo.c​ om
Accepted 8 November 2020

SUMMARY
Carotid artery dissection is one of the most common
causes of ischaemic stroke in young and middle-­aged
population. We report a case of bilateral carotid artery
dissection presenting with opercular syndrome or
Foix-C
­ havany-­Marie syndrome. This 46-­year-­old obese
and hypertensive man with a history of fall from bike
1 week prior, presented with sudden onset of anarthria,
dysphagia and deviation of angle of mouth. His speech
and dysphagia gradually improved over 10 days to
normal, but he developed pseudobulbar affect and
difficulty in calculations 4 weeks later. MRI showed acute
infarcts in bilateral operculum. CT angiography showed
dissection in bilateral cervical ICAs. He was managed
conservatively with oral anticoagulation, given for
6 months. Probability of dissection must be considered in
patients with a history of trauma or falls developing focal
neurological deficits. To our knowledge, this is the first
case report of bilateral ICA dissection presenting with
­ havany-­Marie syndrome.
Foix-C

loss of consciousness. He did not have any visual
disturbance, sensory deficits or limb weakness. His
verbal output and dysphagia gradually improved
over 10 days to normal. However, he developed
uncontrollable outbursts of laughter and difficulty
in calculations for which he visited the outpatient
department after 4 weeks of initial presentation.
His clinical presentation fitted into bilateral opercular syndrome or Foix-­Chavany-­Marie syndrome.
On examination, he had no stigmata of connective tissue disorders or of hypercholesterolemia.
His pulse was normal and felt equally in both the
sides. Cardiac system examination was normal. His
Mini-­Mental State Examination score was 22/30.
He had impairment in attention, calculation and
recall. There was also left seventh nerve palsy and
pseudobulbar affect. His power was normal in all
the limbs, but he had brisk deep tendon reflexes
and flexor plantar response. The examination of
sensory, cerebellar and extrapyramidal systems was
normal.

BACKGROUND

INVESTIGATIONS

Carotid artery dissection, a significant aetiology
of ischaemic stroke is commoner in young and
middle aged people as compared with older.1
Internal carotid artery dissection can be intracranial or extracranial, depending on aetiology and
predisposing factors. It is a multifactorial disease,
with causes being either traumatic or spontaneous. Predisposing factors include hypertension,
hyperhomocysteinemia, connective tissue diseases,
such as Ehler-­Danlos syndrome and fibromuscular
dysplasia.2 3 High index of suspicion is warranted
when carotid dissection is suspected particularly
whenever a patient presents with focal neurologic
signs and symptoms along with a history of major or
minor neck trauma. We present a patient with opercular syndrome and pseudobulbar affect caused by
dissection of bilateral Internal Carotid Arteries and
Middle Cerebral Arteries. Opercular syndrome is a
rare pseudobulbar palsy caused by insult to bilateral
operculum, the most frequent cause being ischemia.

Routine haematological and biochemical investigations including lipid profile and HbA1c were
normal. ECG did not show any atrial fibrillation.
2D echocardiogram showed concentric left ventricular hypertrophy with grade 1 diastolic dysfunction
and normal ejection fraction. Renal artery Doppler
did not show any stenosis. MRI brain showed
acute to subacute infarcts in bilateral frontoparietal lobes in the water-­shed zones (figure 1C–F).
MR angiography (MRA) was suggestive of dissection in bilateral cervical internal carotid arteries
with significant luminal narrowing extending up to
bilateral middle cerebral arteries. CT angiography
(CTA) also revealed similar findings (figure 1A,B).

DIFFERENTIAL DIAGNOSIS

We evaluated for secondary causes of dissection like
connective tissue disorders, but could not find any
underlying cause in our patient.

TREATMENT
CASE PRESENTATION

© BMJ Publishing Group
Limited 2020. No commercial
re-­use. See rights and
permissions. Published by BMJ.
To cite: Singh R, Rebello A,
Prabhat N, et al. BMJ Case
Rep 2020;13:e239080.
doi:10.1136/bcr-2020239080

A 46-­year-­old obese man with essential hypertension diagnosed 3 years ago, developed sudden onset
of loss of speech, dysphagia and deviation of angle
of mouth to the right side. He was a chronic smoker
and had alcohol use disorder. He had a history of
fall from motorbike while riding under the influence of alcohol 1 week prior to the onset of these
symptoms. He did not have headache, neck pain,
vomiting, bleeding from ear, nose or mouth and

He was managed conservatively with oral anticoagulation, which was given for 6 months and antihypertensive drugs were optimised. He was also given
dextromethorphan for his pseudobulbar affect.

OUTCOME AND FOLLOW-UP

On follow-­
up at 6 months, his pseudobulbar
affect and memory disturbance remained the same
without any new symptoms. His anticoagulation
was stopped after 6 months.

Singh R, et al. BMJ Case Rep 2020;13:e239080. doi:10.1136/bcr-2020-239080

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Case report

Figure 1 CT angiography showing dissection in bilateral cervical ICA
extending up to bilateral MCA with significant luminal narrowing (A,B).
MRI of brain showing acute infarcts in bilateral operculum (C–F).

DISCUSSION

Arterial dissection is one of the most common causes of stroke
in young and middle-­aged population. It accounts for 1%–2%
of all ischaemic strokes. In younger patients, it contributes to
10%–24% of the total strokes.4–6 In an Indian study of young
strokes, arterial dissection was the cause in 7% of the cases.7
The pathophysiology of cervical artery dissection is not yet well
understood. It has a multifactorial aetiology which includes
trauma and connective tissue disorders which have risk for spontaneous dissection, such as Ehlers-­Danlos syndrome. Trauma is
an important cause of dissection. Traumatic dissection can occur
as a result of non-­penetrating or penetrating neck trauma.2 Our
patient had a history of fall from bike 1 week prior to the onset
of his symptoms. A diagnosis of carotid artery dissection should
be considered in patients presenting with stroke and a history of
headache, facial pain, neck pain, Horner’s syndrome, tinnitus
and/or cranial nerve palsies. These symptoms may present
immediately after dissection, within minutes to hours or up to
1 month after the dissection. Pain is usually the most common
initial symptom. Some patients may remain asymptomatic for
a prolonged period, like our patient who was asymptomatic
for 1 week after trauma.2 8 Pathologically, carotid dissection is
associated with haematoma in the wall of the artery resulting
in stenosis, aneurysmal dilatation and cerebral ischaemia due
to embolism or haemodynamic infarction.9 Cerebral ischemia
resulting in transient ischaemic attacks or strokes was seen in
56% of the cases of cervical artery dissection in a study.10 There
can be bilateral involvement as in our case in around 5%–10%
of the carotid artery dissections.11 However, bilateral opercular
syndrome due to bilateral carotid artery dissection and the
resulting cerebral ischemia has not been reported in the literature before.
Opercular syndrome or Foix-­
Chavany-­
Marie syndrome is
characterised by dissociation of automatic and voluntary movements. The voluntary control of face, tongue and the pharyngeal muscles is provided by primary motor cortex and pyramidal
tract, whereas the spontaneous and the emotional control is
sub-­served by the thalamus, hypothalamus and extrapyramidal
tract.12 In opercular syndrome, volitional control of the lingual,
pharyngeal, facial and the masticatory musculature is lost with
preserved automatic movements. Clinical manifestations include
difficulty in opening mouth, protruding tongue, chewing, swallowing and speech impairment.12 Opercular syndrome is caused
2

by bilateral opercular cortex lesions, surrounding the insula
although sometimes unilateral lesion may also present with
similar signs and symptoms.13
Opercular syndrome can be acquired (as in our patient) or
congenital, intermittent or persistent. The causes of opercular
syndrome include vascular diseases, bilateral perisylvian cortical
dysplasia, CNS infection, neurodegenerative disorder or sometimes epilepsy. Most commonly reported cases, including our
case, are attributed to occlusion of middle cerebral artery branches
which supply the operculum, by thrombosis or embolism.14 Our
patient had bilateral carotid artery dissection extending up to
bilateral middle cerebral artery which led to opercular syndrome
and recent memory loss. He improved partially with residual
deficits which included pseudobulbar affect and memory loss.
invasively with
Carotid dissection can be diagnosed non-­
MRA, CTA or carotid ultrasound. MRA and CTA are found
to be similar in terms of diagnosing carotid dissection, with
CTA being often preferred. CTA is becoming gold standard for
patients with symptomatic cervical artery dissection.13 15

Learning points
►► There has to be a high index of suspicion of carotid dissection

in patients presenting with neurological dysfunction
after trauma despite the absence of definite neck pain or
headache.
►► Bilateral ICA dissection is a rare cause of bilateral opercular
syndrome, also known as Foix-­Chavany-­Marie syndrome.
►► Opercular syndrome is characterised by dissociation of
automatic and voluntary movements of lingual, pharyngeal,
facial and the masticatory musculature.

Contributors RS and AR drafted the manuscript. NP had a role in critical review
of the manuscript. RS and DK managed the patient and had a role in conception,
organisation and execution of the report.
Funding The authors have not declared a specific grant for this research from any
funding agency in the public, commercial or not-­for-­profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
ORCID iDs
Rajveer Singh http://​orcid.​org/​0000-​0001-​7173-​8035
Alex Rebello http://​orcid.​org/​0000-​0002-​0954-​5475

REFERENCES

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Singh R, et al. BMJ Case Rep 2020;13:e239080. doi:10.1136/bcr-2020-239080

BMJ Case Rep: first published as 10.1136/bcr-2020-239080 on 30 November 2020. Downloaded from http://casereports.bmj.com/ on November 30, 2020 at Librarian Ferriman Information &
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Case report

12 Milanlioglu A, Aydın MN, Gökgül A, et al. Ischemic bilateral opercular syndrome. Case
Rep Med 2013;2013:1–3.
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Case report