Wednesday, December 28, 2011

Bilateral facial nerve palsy

Foix-Chavany-Marie (bi-opercular) syndromeIn 1926, Foix, Chavany, and Marie described an acquired syndrome of faciopharyngoglossomasticatory diplegia, caused by bilateral infarction of the anterior operculum (eg, anterior choroidal artery infarction). Clinical features included: facial diplegia, dysarthria, pseudobulbar palsy, cognitive deficits, and seizures. Foix-Chavany-Marie syndrome is also known as the biopercular syndrome (descriptively based on the typical associated lesions affecting the anterior operculum bilaterally) or faciopharyngoglossomasticatory diplegia with automatic voluntary association (descriptively based on the clinical features). Essentially this is a cortico-subcortical type of suprabulbar palsy.

Clinically there is loss of voluntary movements of the jaw, face, pharynx, and tongue, with preserved automatic and reflex movements. For example, there may be a prominent severe facial diplegia to volitional actions (affecting both the upper and lower face, so-called "pseudoperipheral" facial palsy), with impaired volitional eye closure, inability to close the jaw, dysphagia, anarthria, and inability to protrude the tongue. However, corneal reflexes, jaw jerk, and swallowing reflexes are preserved (although the gag reflex is typically diminished or absent). Also preserved are "automatic" movements, including jaw opening with yawning, blinking (spontaneous or to threat), emotional smile, facial muscle contraction during crying, ability to contract the frontalis muscle with upgaze, and ability to close the eyes during sleep. Other possible neurologic manifestations include distal weakness in the arms, cheiro-oral paresthesias, and Broca aphasia.
Most cases are caused by ischemic strokes, but postsurgical, infectious (eg, Herpes simplex encephalitis, HIV, cerebral toxoplasmosis, etc.), epileptic (typically reversible in childhood epilepsy syndromes), traumatic, developmental (eg, bilateral perisylvian dysgenesis resulting from neuronal migration disorders), and rarely neurodegenerative causes are also recognized. Foix-Chavany-Marie syndrome may be confused with conditions causing bilateral peripheral facial dysfunction (eg, botulism, Guillain-Barré syndrome, or myasthenia).
Heerdfordt syndrome (neurosarcoidosis)Heerdfordt syndrome is a variety of neurosarcoidosis.
Manifestations include:• fever• uveitis• parotid gland swelling (unilateral or bilateral)• facial paresis (unilateral or bilateral)
Localization of the lesions responsible for facial paresis has been controversial. Proposed localizations have included (1) direct facial nerve compression by swollen parotid glands; (2) lesions within the facial canal (based on accompanying taste disturbance in some cases); and (3) lesions of the cerebellopontine angle that spread distally into the facial canal (based on electrical and mechanical stimulation studies and MRI). Treatment with immunosuppressive therapy can relieve symptoms, including facial paresis. Enhancing lesions on MRI may resolve with corticosteroid therapy.
Lyme neuroborreliosisFacial palsy is common in patients with Lyme neuroborreliosis and may occur in up to half of patients with Borrelia burgdorgeri meningopolyradiculoneuritis. It is particularly common in children with Lyme neuroborreliosis. Cases with a unilateral facial palsy and a history of tick bite and/or erythema chronicum migrans in the head/neck region generally have the facial palsy on the same side as the bite, suggesting direct nerve invasion by Borrelia burgdorferi in most unilateral cases. Whether this is also true in bilateral cases is not clear. Facial palsy generally occurs in the setting of meningitis and is often bilateral (about one third of cases with facial paresis due to Lyme disease). Approximately one third of cases are initially complete.
Manifestations can include:• fever• headache• facial paresis (unilateral or bilateral)• CSF pleocytosis and increased protein• MRI enhancement of tentorium and the trigeminal and facial nerves
Oral doxycycline is an effective and convenient therapy for Lyme-disease associated facial paresis. With therapy, 90% recover without sequelae within 6 months. Recovery is probably lower with bilateral facial paresis, but still approximately two thirds recover.
Melkersson-Rosenthal syndrome (OMIM #155900)Melkersson-Rosenthal syndrome (OMIM #155900) is a triad of (1) recurrent facial paresis (unilateral or bilateral); (2) relapsing facial edema, involving especially the lips; and (3) associated fissured tongue (lingua plicata or "scrotal tongue"). The complete triad is present, though, in less than one third of patients. Onset is often in childhood. Residual facial paresis generally increases with each episode and with concomitant development of progressive synkinesias. The gene locus is on 9p11.
Möbius syndrome (OMIM #157900)Möbius syndrome (OMIM #157900) is a syndrome of rhombencephalic maldevelopment involving predominantly brainstem motor nuclei and axons, as well as traversing long tracts. Vascular insufficiency prior to the 6th week of gestation involving the proximal sixth intersegmental artery may be responsible. Most cases are sporadic but inherited forms have been linked to 13q12.2-q13, 3q21-q22, and 10q21.3-q22.1.
The syndromic definition varies somewhat across authors. Some label all cases of congential facial paresis as Möbius syndrome, whereas others restrict it to cases of congenital sixth and seventh nerve paresis with skeletal defects.
Manifestations may include:• facial diparesis• bilateral (or rarely unilateral) ocular abduction paresis• Duane retraction syndrome• congenital fibrosis of ocular muscles• orofacial malformations (eg, cleft palate/cleft lip, microglossia, micrognathia)• paresis of lower cranial nerves (IX-XII), especially XII• absence of pectoralis major muscle (Poland anomaly)• terminal transverse limb defects• diaphragmatic anomalies• poor motor development• poor coordination• feeding difficulties in infancy• drooling• respiratory abnormalities.

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