Foix-Chavany-Marie syndrome

Douglas J Lanska MD FAAN MS MSPH (Dr. Lanska of the Great Lakes VA Healthcare System and the University of Wisconsin School of Medicine and Public Health has no relevant financial relationships to disclose.)
Originally released June 12, 2011; last updated January 28, 2017; expires January 28, 2020

This article includes discussion of Foix-Chavany-Marie syndrome, opercular syndrome, fascio-labial-pharyngo-glosso-laryngo-brachial paralysis with automatic-voluntary dissociation, and pseudobulbar paralysis (cortical type). The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

In this article, the author explains the clinical presentation and pathophysiology of Foix-Chavany-Marie syndrome (bilateral anterior opercular syndrome). Manifestations include volitional paralysis of masticatory, facial, pharyngeal, and lingual muscles innervated by cranial nerves V, VII, IX, X, and XII, with preserved autonomic and emotional innervation of these muscles. Thus, although volitional movements are absent, emotional smiling, laughter, crying, and automatic yawning are preserved. Pseudobulbar paralysis in Foix-Chavany-Marie syndrome must be distinguished from bulbar paralysis and from disorders of the cranial nerves and neuromuscular junction such as botulism and myasthenia gravis.

Key points

 

• Foix-Chavany-Marie syndrome is a rare cortical form of supranuclear (pseudobulbar) palsy caused by bilateral anterior opercular lesions; in this syndrome there is an “automatic-voluntary dissociation” of motor function of lower cranial nerves.

 

• Manifestations include volitional paralysis of masticatory, facial, pharyngeal, and lingual muscles innervated by cranial nerves V, VII, IX, X, and XII, with preserved autonomic and emotional innervation of these muscles (eg, emotional smiling, laughter, and crying as well as automatic yawning).

 

• Pseudobulbar paralysis in Foix-Chavany-Marie syndrome is clinically distinguished from bulbar paralysis and from disorders of the cranial nerves and neuromuscular junction (eg, botulism and myasthenia gravis) by normal eye movements, preserved or hyperactive brainstem reflexes (eg, jaw jerk), the dissociation of automatic and volitional movements of the bulbar muscles with preservation of automatic movements, and the absence of atrophy and fasciculations of the lower motor neuron-innervated muscles.

 

• The cortical form of pseudobulbar palsy is clinically distinguished from the noncortical type of pseudobulbar palsy by its acute onset, prominent voluntary-automatic dissociation of the bulbar muscles, absence of emotional lability, and absence of bowel and bladder incontinence.

 

• There are 5 predominant clinical groups of Foix-Chavany-Marie syndrome (Weller 1993): (1) an acute form, typically with bilateral infarction of the anterior opercula; (2) a subacute form, typically caused by central nervous system infections; (3) a developmental form, attributed to neuronal migration disorders; (4) a reversible form in children with epilepsy; and (5) a rare chronic progressive form associated with neurodegenerative disorders.

Historical note and terminology

The bilateral anterior opercular syndrome was first described by Magnus in 1837 and later more extensively investigated by French neurologists Charles Foix (1882-1927) and Jean Alfred Émile Chavany (1892-1959) with French pediatrician Julien Marie (1899-1987) in 1926 (Foix et al 1926; Foix and Chavany 1926; Magnus 1837).

Image: Charles Foix
These reports are the basis for the eponym Foix-Chavany-Marie syndrome to describe the clinical manifestations of bilateral anterior opercular dysfunction. Although also used, the terms opercular syndrome and anterior opercular syndrome are not sufficiently specific to distinguish Foix-Chavany-Marie syndrome from the very different manifestations of unilateral or posterior opercular lesions.

In the 1950s, a developmental form of the bilateral anterior opercular syndrome was described by Worster-Drought, a consultant neurologist at an institution for children with communication disorders, and this congenital form is sometimes now referred to as the Worster-Drought syndrome (Worster-Drought 1953; Worster-Drought 1956; Worster-Drought 1974; Christen et al 2000), although some use this eponym more loosely to refer to bulbar cerebral palsy (Clark et al 2010). Kuzniecky and colleagues termed the congenital form “congenital bilateral perisylvian syndrome” (Kuzniecky et al 1993). Others have suggested the term “congenital suprabulbar palsy,” but this again is insufficiently specific to distinguish this from subcortical causes of pseudobulbar palsy.

The bilateral anterior opercular syndrome has been more frequently recognized and reported in the international literature since the advent of CT and MRI imaging.

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