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mise à jour le
20 novembre 2003
Eur Neurol
2003;50:185-188
Lexique
Dissociated preservation of automatic-voluntary jaw movements in a patient with biopercular and unilateral pontine infarcts
J. Ghika, F. Vingerhoets, J. Bogousslavsky
Department of Neurology, CHUV, Lausanne, Switzerland

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Diplégie faciale totale avec paralysie glosso-laryngo-cervicale chez deux frères Brissaud, Marie 1893
Syndrome de Foix Chavanny
 
Introduction : Automatic-voluntary dissociation of orofaciolingual-pharyngeal motility is one of the hallmarks of the biopercular Foix-Chavany-Marie syndrome. While automatic-voluntary dissociation of movements of the upper extremities (mostly apraxias) or eyelid movements is well known, similar deficits of other cranial movements have been reported but less well studied. Apraxia is defined as the inability to perform correctly voluntary learned skilled movements upon request in an experimental setting or everyday life (anterior of frontal apraxia), to pantomime (posterior or parietal apraxia), to use objects (on command, imitation or presentation) or to imitate correctly meaningful or meaningless gestures performed by the examiner.
 
Apraxia can result from lesions of the dorsolateral frontoparietal-subcortical circuits interrupting the flow of information at any level between the complex motor programs of the frontal cortical areas and the sensorimotor areas allowing external cues to guide motor behavior. Unfortunately, the definition of apraxia has been extended for apraxia of eyelid opening to a syndromic entity of. In the Foix- Chavany-Marie syndrome, or biopercular syndrome, pseudo-bulbar palsy with central voluntary palsy is reported, preserving automatic and emotional movements. Multiple brain infarcts are not infrequent and may constitute a unique opportunity to study brain function. We happened to observe a patient with Foix-Chavany-Marie syndrome with a previous unilateral pontine infarct which only preserved the jaw reflex but not emotional movements of the lower face or glossopharyngeal reflexes.
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Patient Report
A 71-year-old hypertensive patient with multifocal vascular disease (ischemic cardiopathy, vascular claudication of the legs) and intermittent atrial fibrillation had a history of left pontine infarcts in September 1985. He complained of acute rotatory vertigo and bifrontal headache, and physical examination revealed gait ataxia, left myosis, bilateral gaze-evoked nystagmus, oculomotor ataxia and right spastic sensorimotor deficit. A brain CT was normal except for mild generalized atrophy. Fifteen days later, he suffered from a left sylvian opercular syndrome with right faciobrachial spastic hemiparesis, Broca aphasia and worsening of the right hemiparesis. He recovered with severe dysarthria, a plegic right arm and a stiff right leg, but was able to walk with a cane during the following months.
 
In September 1994, he was rehospitalized for an acute left motor hemiparesis, anarthria, transient conjugate gaze and head deviation to the right and a pseudobulbar syndrome without emotional lability or spasmodic laughing or crying. On physical examination he was slightly drowsy, oriented, and with Cheynes-Stokes respiratory pattern. He had lower face diplegia, severe corticobulbar syndrome with spastic plegia of jaw, trismus, clonic jaw reflex, absent voluntary movements of the tongue, anarthria (aphemia), dysphagia, aphonia, absent gag reflex, bilateral paresis of sternocleidomastoid and trapezius muscles. Orolingual masticatory apraxia could not be tested because of this important paresis, but upper face motility (lids, frontal and eye movements and praxias) were normal but slow. He understood and executed simple and complex orders. We could not see any emotional movements of the lower face.
 
On detailed examination of jaw movement, he could not open or close the jaw on order and needed to help himself with his hand in order to open or close his mouth. When asked to do so, despite strenuous attempts of synkinesia, such as opening of the eyelids, contracting the frontalis muscle, or through movements of the eyes, or retraction of the head backwards, his mouth remained shut. Similarly, he was also totally unable to cough on command. However, the patient was found on several occasions with his mouth wide open and movements of the inferior facial muscles when yawning.
 
Reflex attempts of chewing movements (with decreased amplitude and bulging of the lips) to objects introduced into the mouth, such a straw or a spoon, could be observed, but no deglutition or phonation. Brainstem pontine reflexes such as cochleopalpebral, vestibulo-ocular, and horizontal oculocephalic reflexes, upper face mimicking pain to nose pinprick, corneal reflex and jaw reflex (which was clonic) were all preserved in the upper face only. Bulbar reflexes such as coughing, swallowing, gag reflex and reflex phonation to pain were abolished. Similarly, he was also totally unable to cough, shout or breathe on command. There was a spastic and hyperreflexic asymmetric tetraparesis with a right algotactile hemisensory deficit, with hand and foot clonus and bilateral Babinski signs. A brain CT and MRI with angio-MRI showed an acute right superficial opercular and parietal sylvian infarct, an old contralateral left insular and opercular lesion corresponding to the second stroke and an old left pontine infarct corresponding to the first stroke and small subcortical white matter lesions. MRI angiogram was unremarkable as well as Doppler ultrasound examination of neck and brain vessels. The strokes probably had a cardioembolic origin.
 
Discussion
Our patient presented with a biopercular Foix-Chavany-Marie syndrome and a unilateral pontine lesion with disappearance of normally preserved automatic-emotional/voluntary reflex activity of the lower face, pharynx, larynx and tongue, except for reflex jaw movement.
 
Initiation and control of mandibular movements are processed by neural pathways descending from the lateral sensorimotor facial cortical area via direct and indirect connections, facilitating motoneurones of jaw opening and suppressing excitability of jaw closing motoneurones of the trigeminal motor nucleus.
 
The roles of the supplementary motor area, premotor cortex, the cingulate motor area as well as parietal areas and cerebellum have been studied in detail with PET scan and fMRI for movements of the hand, but have so far not been demonstrated for facial and branchial motor muscle groups.
 
Electrophysiological studies in monkeys showed that corticobulbar neurons are localized in the precentral gyrus and anterior bank of the central sulcus with direct projections to the brainstem. The activity of 75% of the neurons in the jaw movement area was related to opening of the jaw, keeping the jaw open or lateral movements, whereas very few were related to closing movements. Closing of the jaw and rhythmic masticatory movements are probably processed at the segmental level of the brainstem (except for graded or tonic biting), as well as their initiation and ending and the complex coordination of tongue and jaw muscles necessary to chew complex foods, positioning the food bolus, preventing biting lesions or coordinating highly complex speech movements.
 
In humans, transcranial magnetic stimulation of the masseter has been possible, which is compatible with a separate tract from pyramidal fibers. A massive bilateral representation is reported, so that only bilateral lesions are followed by significant deficits in jaw and tongue movements. In contrast to the voluntary motor control of the jaw, most of the reflex jaw movements for mastication, yawning, swallowing and speaking are thought to originate in brainstem pontine motor nuclei and reticular formation.
 
Our patient had bilaterally cortical-subcortical lesions near the operculum and insula, disconnecting those areas from the supplementary motor area, premotor and precentral sensorimotor cortical areas, disrupting the highest-level voluntary motor command of the jaw for voluntary jaw opening and closing, but preserving pontine automatic brainstem reflexes despite a previous unilateral brainstem lesion.
 
This is a unique situation that interestingly demonstrates that a unilateral pontine lesion, in the presence of biopercular suprasegmental lesions, is not sufficient to make the reflex jaw movement disappear, but may require bilateral pontine lesions, probably because of a bilateral representation of intersegmental brachial reticular reflexes.
 
In conclusion, our patient represents a unique case of an isolated preservation of an automatic-voluntary dissociation of jaw movement in the presence of a unilateral pontine lesion, which sheds some light onto the brain network of jaw movements in humans.
 
 
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Diplégie faciale totale avec paralysie glosso-laryngo-cervicale chez deux frères
Eugène Brissaud, Pierre Marie
Le Bulletin Médical
1893;7(96):1081-1084
1893
 
Mouvements involontaires dans les membres paralysés
Pierre Marie et André Léri 1911
 
Eugène Brissaud
Pierre Marie
 
 
syndrome operculaire
syndrome de Foix-Chavany-Marie