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A Case of Foix-Chavany-Marie Syndrome with History of Glioblastoma Showing Partial Recovery
Deeksha Seth1, Musab Eltahir2, Gita Gramizadeh-Naeini1
1Stroke Department, Gloucestershire Hospitals NHS Foundation Trust, Gloucester, UK.
Introduction:
Foix-Chavany-Marie syndrome (FCMS) or bilateral opercular syndrome (OPS) is a rare pseudobulbar palsy characterized by facial, lingual, pharyngeal, and masticatory voluntary muscle paralysis resulting in anarthria with preservation of autonomic, involuntary, and reflexive functions. Damage to the posterior part of the inferior frontal gyrus and inferior part of precentral gyrus play a role in the pathogenesis of FCMS.
Case Description:
We report a rare case of bilateral OPS following an acute right middle cerebral artery (MCA) infarct in a patient with history of glioblastoma, and resection in the left MCA territory within the cingulate gyrus, showing recovery of speech and swallowing despite intensive bilateral opercular lesions owing to extensive multidisciplinary team support. However, the patient was discharged with a percutaneous endoscopic gastrostomy tube for long-term enteral feeding support due to partial recovery.
Discussion:
The patient's history of glioblastoma, left MCA cingulate gyrus resection and right MCA infarction with automatic-voluntary dissociation led to the diagnosis of FCMS. Rehabilitation surprisingly showed mild improvement in speech and swallowing despite extensive bilateral opercular lesions proving that there are still chances of improvement in speech and swallowing in OPS with the right multidisciplinary approach.
Conclusion:
Patients with a history of brain tumours like glioblastoma can develop bilateral OPS later in life in case of other vascular events that cause lesions in a previously unaffected operculum, triggering symptoms. Extensive involvement of the speech and language team is of significant in the management of FCMS cases as above where some recovery might still be seen.
Learning Points:
Foix-Chavany-Marie syndrome (FCMS) can be caused by bilateral opercular lesions (new or old or a combination of both) in patients with history of high-grade brain tumours.Patients with bilateral opercular syndrome experience full or partial or no recovery at all of speech and swallowing, hence proper rehabilitation with a Speech and Language Team is significant.Understanding automatic-voluntary dissociation in FCMS and being able to differentiate it from bulbar palsy and other similar phenomena is crucial in making a diagnosis of FCMS.
Insights
Foix-Chavany-Marie syndrome (FCMS), a rare pseudobulbar palsy, can occur in brain tumor patients after vascular events. Multidisciplinary rehabilitation offers potential for speech and swallowing recovery despite bilateral opercular lesions.
Area of Science:
- Neurology
- Neuroscience
- Rare Diseases
Background:
- Foix-Chavany-Marie syndrome (FCMS), also known as bilateral opercular syndrome (OPS), is a rare pseudobulbar palsy.
- It is characterized by voluntary muscle paralysis in the face, tongue, pharynx, and mastication, leading to anarthria while preserving autonomic functions.
- Pathogenesis involves damage to the posterior inferior frontal gyrus and inferior precentral gyrus.
Purpose of the Study:
- To report a rare case of bilateral OPS following an acute right middle cerebral artery (MCA) infarct in a patient with a history of glioblastoma.
- To highlight the role of multidisciplinary team support in achieving partial recovery of speech and swallowing.
- To emphasize the potential for improvement in OPS cases with appropriate rehabilitation.
Main Methods:
- Case report of a patient with a history of glioblastoma and left MCA territory resection.
- Diagnosis of FCMS based on history, MCA infarct, and automatic-voluntary dissociation.
- Assessment of recovery through speech and swallowing function evaluation during rehabilitation.
Main Results:
- The patient presented with bilateral opercular lesions after an MCA infarct, leading to FCMS diagnosis.
- Despite extensive lesions, the patient showed mild improvement in speech and swallowing with multidisciplinary support.
- The patient was discharged with a feeding tube due to partial recovery, underscoring the need for long-term support.
Conclusions:
- FCMS can develop in patients with a history of brain tumors like glioblastoma following subsequent vascular events.
- Effective rehabilitation, particularly with a Speech and Language Team, is crucial for managing FCMS and optimizing recovery.
- Differentiating FCMS from other conditions like bulbar palsy is essential for accurate diagnosis and treatment.
