Foix-Chavany-Marie Syndrome as a Manifestation of Unilateral Opercular Stroke
Roberto Toledo-Trevino1, Diana Manrique-Otero1, Enrique Castellanos-Pedroza1, Vanessa Cano-Nigenda1, Alonso Alvarado-Bolaños1, Juan Calleja-Castillo2, Antonio Arauz1
1 Stroke Clinic, National Institute of Neurology and Neurosurgery Manuel Velasco Suárez, Mexico City, Mexico
2 Emergency Department, National Institute of Neurology and Neurosurgery Manuel Velasco Suárez, Mexico City, Mexico
Doi: 10.12890/2021_002462- European Journal of Case Reports in Internal Medicine - © EFIM 2021
Received: 08/03/2021
Accepted: 15/03/2021
Published: 06/04/2021

How to cite this article: Toledo-Trevino R, Manrique-Otero D, Castellanos-Pedroza E, Cano-Nigenda V, Alvarado-Bolaños A, Calleja-Castillo J, Arauz A. Foix-Chavany-Marie syndrome as a manifestation of unilateral opercular stroke. EJCRIM 2021;8 doi:10.12890/2021_002462.

Conflicts of Interests: The authors declare there are no competing interests.
Acknowledgements: We thank our patient for allowing us to report his case.
This article is licensed under a Commons Attribution Non-Commercial 4.0 License

ABSTRACT

Introduction: Foix-Chavany-Marie syndrome (FCMS) is a type of pseudobulbar palsy that affects facio-pharyngo-glosso-masticatory muscles.
Materials and Methods: A 62-year-old man was admitted to the emergency department after 9 hours of acute dysarthria and dysphagia. MRI showed restricted diffusion in the right operculum on diffusion-weighted imaging (DWI). No thrombolytic therapy was given. The patient had a history of mechanical aortic valve replacement under anticoagulation with a vitamin K antagonist. Work-up demonstrated suboptimal levels of INR. Due to severe dysphagia during hospitalization, a percutaneous endoscopic gastrostomy (PEG) was performed.
Results: The patient was discharged 5 days later, with a modified Rankin scale (mRs) score of 3, and secondary stroke prevention. He had achieved an excellent functional outcome (mRs 1) at 6-month follow-up.
Conclusion: Our patient had a satisfactory recovery due to prompt diagnosis, secondary stroke prevention, and compliance with treatment.

LEARNING POINTS

KEYWORDS

Foix-Chavany-Marie, pseudobulbar palsy, opercular syndrome, opercular stroke

INTRODUCTION

Foix-Chavany-Marie syndrome (FCMS) or opercular syndrome is an uncommon type of pseudobulbar palsy where bilateral damage to the operculum generates facio-pharyngo-glosso-masticatory paralysis with automatic-voluntary dissociation as a distinctive feature.
There are three main types of pseudobulbar palsy according to the site of the lesion: cortical, basal ganglia/capsular, and brainstem. FCMS corresponds to the cortical type and unlike the others, rarely presents with emotional incontinence, urinary dysfunction, or muscle tone abnormalities [1].
It is important to consider this syndrome due to cerebrovascular disease as a possible aetiology in patients with acute onset of bilateral facial-pharyngo-glosso-masticatory paralysis. Here, we present the case of a patient with unilateral opercular stroke and FCMS.

CASE DESCRIPTION

A 57-year-old man with a history of adenocarcinoma of unknown primary origin with metastases to the bone, liver, lungs and brain presented with an acute right lower extremity deep vein in-stent thrombosis. Previously, approximately a year ago, he was found to have high-grade stenosis of the distal right external iliac vein from a large tumour in the right hemipelvis which was stented, and he was started on apixaban. His systemic disease responded well to 11 cycles of carboplatin/paclitaxel and pelvic radiation, but unfortunately, 1 month before the presentation, he developed a right frontal lobe brain metastasis requiring whole brain radiation. He was started on levetiracetam 500 mg and dexamethasone 4 mg twice daily for seizure prophylaxis. His cell counts were within the normal range at the time of initiation of the medication, but on presentation, his platelet count had fallen to 35,000/µl. His white blood cell count (WBC) and haemoglobin were 11,000/µl and 15.1 g/dl, respectively (Table 1. His thrombocytopenia was initially attributed to platelet consumption secondary to the thrombus formation. The peripheral blood smear showed no giant platelets, schistocytes, immature cell lines or platelet clumping. His last cycle of chemotherapy was 2 months prior to presentation. Heparin-induced thrombocytopenia (HIT) was unlikely as his platelet count was low before the initiation of heparin this admission. Haptoglobin, vitamin B12, folate, lactate dehydrogenase, liver function tests and total bilirubin were all normal.

METHODS AND PROCEDURES

MRI showed an area of restricted diffusion in the right operculum on diffusion-weighted imaging (DWI), suggestive of an acute ischaemic stroke. T2 FLAIR showed previous silent bilateral parietal infarcts with evidence of small-vessel disease. The brainstem had no lesions and there was no evidence of large-vessel occlusion. No thrombolytic treatment was given as the patient was outside the reperfusion therapeutic window. INR at admission was below therapeutic goals (<2). The rest of the work-up was normal: no arrhythmia was found on Holter monitoring or electrocardiogram, there were no intracavitary thrombi on echocardiogram, and carotid Doppler ultrasound was unremarkable. We concluded that cardioembolic infarction in a patient under suboptimal anticoagulation was the most feasible aetiology. As a result of severe dysphagia during hospitalization, a PEG was performed. The patient had a favourable evolution and was discharged 5 days later under oral anticoagulation and ambulatory speech and swallowing therapy. He had a modified Rankin scale (mRs) score of 3. Six months later, the patient exhibited mild dysarthria, right central facial palsy, no need for a gastrostomy tube, and excellent functional outcome with an mRS of 1.

DISCUSSION

FCMS was described for the first time by Magnus in 1837 and detailed by Foix et al. in 1926. It is the result of bilateral cortical damage to the anterior operculum, which contains motor neurons for the cranial nerves V, VII, IX, X and XII [2]. There are few reports of unilateral damage to the anterior operculum generating FCMS, and it is believed that the presence of asynchronous contralateral corticonuclear tract lesions is the substrate of this unusual presentation [3]. There are also reports where opercular and contralateral damage to the corona radiata or brainstem causes FCMS [1].
FCMS presents as a pseudobulbar palsy with ‘autonomic voluntary dissociation’ where involuntary reflex motor movements, like crying, are preserved [4]. The neuroanatomical basis of this phenomenon is explained by the presence of alternative autonomic pathways between the amygdala and motor cranial nerve nuclei interneurons located in the pontine lateral tegmentum [5]. In our case, the patient presented with an acute ischaemic stroke in the right operculum evidenced on DWI (Fig. 1) and previous left subcortical and periventricular lesions seen on T2 FLAIR (Fig. 2), which explains the existence of contralateral damage to corticonuclear tracts and thereby the presence of FCMS. In the presence of acute bilateral facial-pharyngo-glosso-masticatory paralysis, stroke should be considered as the most feasible aetiology and FCMS syndrome as a possible underlying neuroanatomical explanation. Cerebrovascular disease is the most common cause of FCMS, but there are reports with central nervous system inflammation, trauma, neurodegenerative disorders, epileptic activity, or vascular malformations as the aetiology [4].

Figure 1 (click to enlarge)

Figure 2 (click to enlarge)

Figure 1. MRI with restriction in the diffusion-weighted imaging (DWI) sequence showing an acute right opercular infarction, as well as a previous infarction in the posterior parietal region
Figure 2. MRI shows positive diffuse bilateral lesions in the periventricular and subcortical white matter on the T2 FLAIR sequence

The treatment and prognosis of FCMS are related to the aetiology. Usually, patients experience poor recovery. Airway protection and gastrostomy are cornerstones in acute settings, as patients have a significantly higher risk of developing aspiration pneumonia. Swallowing and speech therapy play an essential role in rehabilitation. Treatment of the underlying cause is the main therapeutic action, such as secondary stroke prevention [5]. Even though our patient had several comorbidities, he experienced good recovery and prognosis mainly due to prompt diagnosis and excellent treatment compliance.

CONCLUSION

LEV-induced bone marrow suppression is a rare but important differential to consider in patients with new-onset isolated thrombocytopenia or pancytopenia. It may become difficult to diagnose the condition especially when other differential diagnoses also need to be equally considered. As most cases occur within the first month of initiation of the medication, it may be prudent to check a complete blood count during this time frame and follow patients closely if a drop in cell counts is seen.

References
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