Foix-Chavany-Marie syndrome, also known as bilateral anterior opercular syndrome or anterior operculum syndrome, is a cortical form of pseudobulbar palsy classically caused by bilateral anterior opercular lesions.
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Clinical presentation
The clinical presentation is of pseudobulbar palsy that is usually acute 1,2, although the tempo of the condition can vary depending on its etiology 1-11. There is paralysis of bilateral 1,2:
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masticatory muscles
causing an inability to masticate or close the jaw
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facial muscles, in a pseudo-lower motor neuron pattern
causing a complete facial diplegia
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pharyngeal muscles
causing dysphagia
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lingual muscles
causing dysarthria or anarthria
The facio-pharyngo-glosso-masticatory diplegia is typically flaccid and only of voluntary action, while automatic actions (e.g. laughter, yawning) remain intact, known as automatic-voluntary dissociation 1,2. Sensory functions also remain preserved, while the gag reflex is typically absent 1,2.
Additionally, unlike other non-cortical causes of pseudobulbar palsy, there is no pseudobulbar affect present 2. Notably, in congenital forms of Foix-Chavany-Marie syndrome, there may be associated intellectual impairment and epilepsy 11.
Pathology
Foix-Chavany-Marie syndrome is typically caused by bilateral pathology affecting the anterior frontal operculum, which is adjacent to and has connections with the region of the primary motor cortex responsible for voluntary action of the masticatory, facial, pharyngeal and lingual muscles 1,2. Automatic action is thought to be preserved because it is mediated by neurons located away from the anterior operculum, likely in other pathways between the brainstem, amygdala and lateral hypothalamus 3.
Etiology
There are many causes, which can be divided by the resultant tempo of the clinical presentation.
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acute:
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ischemic stroke, typically sequential but rarely simultaneous or only unilateral 1,2,4
most common cause
other: traumatic brain injury, intracerebral hemorrhage, demyelination, etc. 5,6
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subacute: infections (e.g. HSV encephalitis, cerebral abscess) 7,8
chronic: neurodegenerative disorders 9,10
congenital: bilateral perisylvian polymicrogyria, Worster-Drought syndrome 11
Radiographic features
Radiographic features are best seen on MRI brain but will depend on the underlying etiology of Foix-Chavany-Marie syndrome.
Treatment and prognosis
Management depends on the underlying etiology, but may incorporate supportive measures such as nasogastric feeding and speech pathology input.
History and etymology
The syndrome is named after Charles Foix (1882-1927), Jean Alfred Chavany (1892-1959) and Julien Marie (1899-1987), French physicians, who described the condition in detail in 1926 12. However, the clinical features of Foix-Chavany-Marie syndrome were first described by A Magnus many years earlier in 1837 13.
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