Opercular syndrome (Foix–Chavany–Marie syndrome) secondary to corona radiata stroke and polymicrogyria: A case report

INTRODUCTION

Foix–Chavany–Marie syndrome (FCMS) was initially described by Magnus in the 19^th century and further defined by Foix, Chavany, and Marie in 1926, hence the name of the disease. Anarthia and bilateral facio-linguovelopharyngeo-masticatory volitional paralysis with preserved reflexive facial movements are the clinical hallmark features of this condition.^[1] Cerebrovascular disease is the most common cause; other important causes include central nervous system infections, neuronal migration disorders, and neurodegenerative conditions.^[1] Although FCMS was rarely reported in the past, the advent of magnetic resonance imaging (MRI) has now led to prompt diagnosis and treatment.

Here, we discuss the case of a middle-aged man with an underlying asymptomatic chronic unilateral brain lesion clinically decompensating on the occurrence of an acute contralateral ischemic stroke to manifest a disconnection syndrome. This report emphasizes the need to look for clinically unapparent chronic brain lesions in the neuroimaging of a patient presenting with anarthria and dysphagia, which could contribute to accurately diagnosing FCMS.

CASE REPORT

Patient presentation and initial investigations

A 61-year-old male with a history of cerebral palsy, epilepsy, and paranoid personality disorder presented with the abrupt onset of cessation of speech output and difficulty swallowing. He had no prior focal neurological deficits, and there was no history of trauma or recent illness. On neurological examination, he was aphonic, but his verbal comprehension and reading perceptions were intact. He was able to answer simple questions with signs and writing. He was unable to open his mouth, protrude his tongue, show his teeth, chew, or swallow. Emotional facial expressions and reflexive movements of the face, such as smiling and yawning, were preserved as validated using observations made and documented by healthcare professionals during the patient’s interaction with his relatives and his facial response to funny matters. The limb muscle strengths were 5/5, deep tendon reflexes were normoactive, and coordination was normal, ruling out widespread motor involvement.

Initial investigations included routine blood tests, inflammatory markers, and cerebrospinal fluid analysis, which were unremarkable [Table 1]. A brain MRI showed a left-sided corona radiata infarct and chronic right opercular polymicrogyria [Figures 1 and 2] suggesting an effective potential disruption of the left and the right corticobulbar pathways, respectively, raising suspicion of a rare neurologic syndrome named FCMS.

Table 1: Lab investigations.

Investigation	Result	Reference range
Full blood count	9.0	3.6–11.0×109/L
Urea	10.7	2.5–7.8 mmol/L
Sodium	144	133–146 mmol/L
Potassium	4.1	3.5–5.3 mmol/L
C-reactive protein	14 mg/L	0.1–5 mg/L
Liver function tests	Alkaline phosphatase 141 IU/L	30–130 IU/L
HbA1c	33 mmol/mol	<42 mmol/mol
Plasma viscosity	1.70 mPa.s	1.48–1.72 mPa.s
Thyroid stimulating hormone	1.3 mIU/L	0.35–4.94 mIU/L
c-ANCA/p-ANCA	Negative
Serum protein electrophoresis	Negative
Antiganglioside antibodies	Negative
Antinuclear antibody	Negative
5-day Holter monitor study	Normal

c-ANCA: Cytoplasmic pattern of anti-neutrophil cytoplasmic antibody. p-ANCA: Perinuclear pattern of anti-neutrophil cytoplasmic antibody.

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Figure 1:

61-year-old man with Foix-Chavany-Marie syndrome who presented with anarthria and dysphagia. (a) The diffusion-weighted image (B1000) shows restricted diffusion in the left corona radiata (purple arrow). (b) The corresponding apparent diffusion coefficient map shows restricted diffusion in the left corona radiata (purple arrow) in keeping with an acute infarct.

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Figure 2:

61-year-old man with Foix-Chavany-Marie syndrome who presented with anarthria and dysphagia. (a) The T2W image reveals extensive right frontal lobe cortical malformation with polymicrogyria (green arrow). (b) The T2W image shows the right frontal lobe cortical malformation with polymicrogyria extending to involve the right frontal operculum (green arrows).

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DISCUSSION

FCMS results from disruptions in the corticobulbar pathways that control voluntary orofacial movements. The most common cause is bilateral opercular damage due to etiologies including ischemic or hemorrhagic strokes, traumatic brain injury, infections (such as encephalitis or abscesses), congenital conditions such as perisylvian polymicrogyria or Worster– Drought syndrome, tumors, and demyelinating diseases such as multiple sclerosis. These diverse causes highlight the need for careful evaluation of both acute and chronic structural lesions when assessing patients with orofacial motor deficits.

The hallmark clinical features of FCMS include profound loss of voluntary control over facial, lingual, and pharyngeal muscles, leading to anarthria and dysphagia. Reflexive and emotional movements, such as smiling or crying, remain intact because these actions are mediated by subcortical circuits, such as those involving the amygdala, hypothalamus, and striatum [Figure 3]. A unilateral lesion disrupting the corticobulbar pathways often produces only mild deficits due to the bilateral corticobulbar innervation of cranial motor nuclei.^[4]

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Figure 3:

A schematic diagram representing the pathophysiology leading to “automatic (involuntary)-voluntary dissociation,” a characteristic clinical phenomenon seen in Foix–Chavany–Marie syndrome.

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Although bilateral opercular damage is the most common cause, this case demonstrates how unilateral lesions (ischemic stroke - in this case), combined with pre-existing asymptomatic contralateral abnormalities (polymicrogyria - in this case), can produce similar symptoms.^[5,6]

Early recognition of FCMS is crucial to avoid unnecessary treatments, particularly in distinguishing it from mimics such as pseudobulbar palsy, GBS, or MG. Misdiagnosis could lead to inappropriate use of immunosuppressive therapies, such as steroids or intravenous immunoglobulin. In this case, the preserved reflexive movements and imaging findings were critical in identifying the disconnection syndrome and steering the diagnostic process away from inflammatory or autoimmune etiologies.

Although the prognosis for FCMS remains poor, early diagnosis allows clinicians to implement targeted interventions, including secondary prevention if it is due to a stroke, nutritional support through feeding tubes, and multidisciplinary rehabilitation.

CONCLUSION

This case highlights the diagnostic challenges of FCMS and the importance of recognizing rare presentations of disconnection syndromes. The combination of a left corona radiata infarction with contralateral opercular polymicrogyria causes a functional bilateral opercular syndrome.

Early and accurate identification of FCMS is crucial to avoid unnecessary treatments. This case highlights the role of detailed neurological examination and neuroimaging in identifying underlying structural lesions contributing to neurological dysfunction. The significance of dual-lesion mechanisms in rare neurological syndromes has been highlighted through our case, demonstrating how congenital abnormalities can become clinically relevant when compounded by an acute vascular event.

Although FCMS has been well-reported, increased awareness of FCMS among clinicians can aid in earlier recognition, accurate diagnosis, and appropriate rehabilitation strategies to optimize patient outcomes. Further research into rehabilitation approaches and functional recovery in FCMS is warranted to improve long-term prognoses.