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Year : 2021  |  Volume : 69  |  Issue : 5  |  Page : 1463--1464

Foix-Chavany-Marie Syndrome - A Rare Presentation of Unilateral Opercular Infarction

Abhishek Juneja, Kuljeet S Anand, Jyoti Garg, Arpit Agrawal 
 Department of Neurology, Dr RML Hospital, Delhi, India

Correspondence Address:
Abhishek Juneja
A-15, Old Quarters, Ramesh Nagar, New Delhi - 110 015

How to cite this article:
Juneja A, Anand KS, Garg J, Agrawal A. Foix-Chavany-Marie Syndrome - A Rare Presentation of Unilateral Opercular Infarction.Neurol India 2021;69:1463-1464

How to cite this URL:
Juneja A, Anand KS, Garg J, Agrawal A. Foix-Chavany-Marie Syndrome - A Rare Presentation of Unilateral Opercular Infarction. Neurol India [serial online] 2021 [cited 2022 Jan 21 ];69:1463-1464
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We report a case of a 60-year-old right-handed male patient who presented with a sudden inability to speak and swallow. He was unable to move his tongue as well as had difficulty in chewing food. He had diabetes mellitus and hypertension for the last 15 years. On physical examination, he was conscious, alert, and anarthric. However, he could comprehend verbal and written commands well. The visual acuity, pupillary light reflexes, extra-ocular movements, and bilateral fundi were normal on examination. Facial sensations and jaw jerks were normal. He had left-sided central facial palsy. Involuntary facial movements were spared. Gag reflex was present with uvula in mid-line. His mouth was half-open, and he could not close it on verbal command. However, mouth closing was noticed during smiling and yawning. There was no tongue atrophy or fasciculation. He could not protrude his tongue out. The rest of the neurological examination was unremarkable. Magnetic resonance imaging (MRI) of the brain revealed right perisylvian infarct [Figure 1]. MRI angiogram of head and neck vessels was normal. The electrocardiogram showed the left bundle branch block. Echo-cardiogram showed global left ventricular hypokinesia with reduced ejection fraction. He was started on antiplatelet and hypolipidemic drugs. A nasogastric tube was inserted for feeding. The patient was following up in the neurology outpatient department without any significant improvement over 4 weeks follow-up.{Figure 1}

Foix-Chavany-Marie Syndrome (FCMS) is a cortical type of pseudobulbar palsy characterized by loss of voluntary control over facial, pharyngeal, lingual, and masticatory muscles. While the reflexive and automatic functions remain preserved leading to automatic–voluntary dissociation. The anatomic basis for such a dissociation has been linked to the presence of alternate pathways connecting the amygdala and hypothalamus to the brainstem controlling reflexive and automatic muscle action.[1],[2]

FCMS is most commonly secondary to bilateral sequential anterior opercular or subcortical insular infarcts.[3] However, unilateral anterior operculum lesions and isolated pontine infarcts causing FCMS have also been reported.[2],[4] This case is unique not only because of unilateral opercular involvement but also because of right-sided perisylvian infarct causing FCMS in a right-handed patient. It may be explained by the hypothesis that contralateral subcortical lesions, which may or may not be visible in an MRI scan, interrupt the projections from the anterior opercular cortex causing FCMS.[2] The prognosis tends to be poor in FCMS with bilateral lesions causing persistent anarthria and dysphagia.[5] Our patient, despite having a unilateral opercular infarct, had severe dysphagia even after 4 weeks of intensive rehabilitation. In conclusion, FCMS should be considered in a patient presenting with acute onset anarthria with automatic–voluntary movement dissociation.

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