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Published on: August 11, 2023
Atypical Presentations of Foix-Chavany-Marie Syndrome (FCMS) in Stroke
Vivek Sanker1, Aariya Srinivasan2, Mohamed Emara3
1General Surgery, Noorul Islam Institute of Medical Science (NIMS), Trivandrum, IND.
Insights
Foix-Chavany-Marie syndrome (FCMS) can occur without the typical bilateral opercular brain lesions. This neurological disorder, characterized by facial, tongue, and throat paralysis, can arise from diverse cerebrovascular events.
Area of Science:
- Neurology
- Neuroscience
- Clinical Medicine
Background:
- Foix-Chavany-Marie syndrome (FCMS), also known as bilateral (B/L) anterior operculum syndrome, is defined by anarthria and central paralysis of facial, lingual, palatal, and masticatory muscles.
- Cerebrovascular disease is the most frequent etiology, but FCMS can also result from CNS infections, developmental disorders, epilepsy, and neurodegenerative conditions.
Observation:
- This report details two atypical FCMS cases in adult males with hypertension, diabetes, and smoking history.
- Both patients presented with acute onset of FCMS symptoms following prior hemiplegia.
- Brain imaging revealed infarcts in the perisylvian region and internal capsule, but notably lacked the classical bilateral opercular lesions.
Findings:
- The presented cases demonstrate that FCMS can manifest without the expected bilateral opercular lesions.
- One patient even presented without any unilateral opercular lesion, challenging established diagnostic criteria.
- The clinical diagnosis of FCMS was confirmed by bifacial, lingual, and pharyngolaryngeal palsy in both individuals.
Implications:
- These findings suggest that the anatomical basis for FCMS is broader than previously understood, extending beyond the opercular regions.
- Clinicians should consider FCMS in the differential diagnosis of anarthria and central orofacial paralysis, even in the absence of typical imaging findings.
- Further research is warranted to fully elucidate the neuroanatomical substrates and etiological spectrum of FCMS.
Abstract:
Foix-Chavany-Marie syndrome (FCMS) presents with anarthria and bilateral (B/L) central facio-linguo-velo-pharyngo-masticatory paralysis with "autonomic voluntary dissociation." The most common cause of FCMS is cerebrovascular disease, while rarer causes include central nervous system infection, developmental disorders, epilepsy, and neurodegenerative disorders. Even though this syndrome is also referred to as (B/L) anterior operculum syndrome, patients with lesion in sites other than (B/L) opercular regions also can develop the syndrome. In this article we describe two such atypical cases. Case 1: A 66-year-old man with diabetes and hypertension who is a smoker had right-sided hemiplegia one year back developed the syndrome acutely two days before admission. CT brain showed left perisylvian infarct and right internal capsule anterior limb infarct. Case 2: A 48-year-old gentleman, who is a diabetic and hypertensive had right-sided hemiplegia one year back and developed the syndrome acutely two days before admission. CT brain showed (B/L) infarcts in the posterior limb of the internal capsule. Both patients had bifacial, lingual, and pharyngolaryngeal palsy thereby confirming the diagnosis of FCMS. None of them had the classical (B/L) opercular lesions on imaging and one patient did not even have a unilateral opercular lesion. Contrary to the common teaching, (B/L) opercular lesions are not always necessary to produce FCMS and can occur even without opercular lesions at all.
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