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.
References:
Arias G, Nogues J, Manos M, Amilibia E, Dicenta M. Bilateral facial nerve palsy: four case reports. ORL J Otorhinolaryngol Relat Spec 1998;60:227-9.
Buyukavci M, Tan H, Akdag R. An alarming sign for serious diseases in children: bilateral facial paralysis. Pediatr Neurol 2002;27:312-3.
Keane JR. Bilateral seventh nerve palsy: analysis of 43 cases and review of the literature. Neurology 1994;44:1198-202.
Wormald PJ, Sellars SL, de Villiers JC. Bilateral facial nerve palsies: Groote Schuur Hospital experience. J Laryngol Otol 1991;105:625-7.
Bell palsy:Adour K, Wingerd J, Doty HE. Prevalence of concurrent diabetes mellitus and idiopathic facial paralysis (Bell's palsy). Diabetes 1975;24:449-51.
McGregor JA, Guberman A, Amer J, Goodlin R. Idiopathic facial nerve paralysis (Bell's palsy) in late pregnancy and the early puerperium. Obstet Gynecol 1987;69:435-8.
Price T, Fife DG. Bilateral simultaneous facial nerve palsy. J Laryngol Otol 2002;116:46-8.
Ralli G, Magliulo G. Bell's palsy and its recurrences. Arch Otorhinolaryngol 1988;244:387-90.
Sartoretti-Schefer S, Kollias S, Wichmann W, Valavanis A. T2-weighted three-dimensional fast spin-echo MR in inflammatory peripheral facial nerve palsy. AJNR Am J Neuroradiol 1998;19:491-5.
Shaikh ZA, Bakshi R, Wasay M, Dai A, Gosy E. Magnetic resonance imaging findings in bilateral Bell's palsy. J Neuroimaging 2000;10:223-5.
Sherwen PJ, Thong NC. Bilateral facial nerve palsy: a case study and literature review. J Otolaryngol 1987;16:28-33.
Stahl N, Ferit T. Recurrent bilateral peripheral facial palsy. J Laryngol Otol 1989;103:117-9.
Congenital:Baek SK, Chae SW, Jung HH. Congenital internal auditory canal stenosis. J Laryngol Otol 2003;117:784-7.
Bergstrom L, Baker BB. Syndromes associated with congenital facial paralysis. Otolaryngol Head Neck Surg 1981;89:336-42.
Carr MM, Ross DA, Zuker RM. Cranial nerve defects in congenital facial palsy. J Otolaryngol 1997;26:80-7.
Shah UK, Ohlms LA, Neault MW, et al. Otologic management in children with the CHARGE association. Int J Pediatr Otorhinolaryngol 1998;44:139-47.
Sjogreen L, Andersson-Norinder J, Jacobsson C. Development of speech, feeding, eating, and facial expression in Mobius sequence. Int J Pediatr Otorhinolaryngol 2001;60:197-204.
St Charles S, DiMario FJ Jr, Grunnet ML. Mobius sequence: further in vivo support for the subclavian artery supply disruption sequence. Am J Med Genet 1993;47:289-93.
Tellier AL, Cormier-Daire V, Abadie V, et al. CHARGE syndrome: report of 47 cases and review. Am J Med Genet 1998;76:402-9.
Verzijl HT, van der Zwaag B, Cruysberg JR, Padberg GW. Mobius syndrome redefined: a syndrome of rhombencephalic maldevelopment. Neurology 2003;61:327-33.
Foix-Chavany-Marie syndrome (Bi-opercular syndrome):Ann MY, Liu OK, Wu YL. Foix-Chavany-Marie syndrome. Zhonghua Yi Xue Za Zhi (Taipei) 2001;64:540-4.
Asenbauer B, McEntagart M, King MD, Gallagher P, Burke M, Farrell MA. Chronic active destructive herpes simplex encephalitis with recovery of viral DNA 12 years after disease onset. Neuropediatrics 1998;29:120-3.
Becker PS. Developmental Foix-Chavany-Marie syndrome: polymicrogyria or macrogyria? Ann Neurol 1990;27:693-4.
Becker PS, Dixon AM, Troncoso JC. Bilateral opercular polymicrogyria. Ann Neurol 1989;25:90-2.
Biller J, Asconape J, Challa VR, Toole JF, McLean WT. A case for cerebral thromboangiitis obliterans. Stroke 1981;12:686-9.
Broussolle E, Bakchine S, Tommasi M, et al. Slowly progressive anarthria with late anterior opercular syndrome: a variant form of frontal cortical atrophy syndromes. J Neurol Sci 1996;144:44-58.
Cellerini M, Mascalchi M, Salvi F, Muscas GC, Dal Pozzo G. MRI of congenital Foix-Chavany-Marie syndrome. Pediatr Radiol 1995;25:316-7.
Christen HJ, Hanefeld F, Kruse E, Imhauser S, Ernst JP, Finkenstaedt M. Foix-Chavany-Marie (anterior operculum) syndrome in childhood: a reappraisal ofWorster-Drought syndrome. Dev Med Child Neurol 2000;42:122-32.
Colamaria V, Sgro V, Caraballo R, et al. Status epilepticus in benign rolandic epilepsy manifesting as anterior operculum syndrome. Epilepsia 1991;32:329-34.
Duffau H, Karachi C, Gatignol P, Capelle L. Transient Foix-Chavany-Marie syndrome after surgical resection of a right insulo-opercular low-grade glioma: case report. Neurosurgery 2003;53:426-31.
Foix C, Chavany JA. Diplégie faciales (facio-linguo-pharingo-masticatrices), d'origine corticale, avec quelques considerations sur les paralysies pseudo-bulbaires et la localization des centres corticaux de l'estrémité céphalique. Ann Med 1926;20:480-98.
Foix C, Chavany JA, Marie J. Diplégie facio-linguo-masticatrice d'origine cortico-suous-cortical sans paralysie des members. Rev Neurol 1926;33:214-9.
Graff-Radford NR, Bosch EP, Stears JC, Tranel D. Developmental Foix-Chavany-Marie syndrome in identical twins. Ann Neurol 1986;20:632-5.
Grattan-Smith PJ, Hopkins IJ, Shield LK, Boldt DW. Acute pseudobulbar palsy due to bilateral focal cortical damage: the opercular syndrome of Foix-Chavany-Marie. J Child Neurol 1989;4:131-6.
Gropman AL, Barkovich AJ, Vezina LG, Conry JA, Dubovsky EC, Packer RJ. Pediatric congenital bilateral perisylvian syndrome: clinical and MRI features in 12 patients. Neuropediatrics 1997;28:198-203.
Hevner RF, Horoupian DS. Pena-Shokeir phenotype associated with bilateral opercular polymicrogyria. Pediatr Neurol 1996;15:348-51.
Iannetti P, Raucci U, Basile LA, et al. Benign epilepsy of childhood with centrotemporal spikes and unilateral developmental opercular dysplasia. Childs Nerv Syst 1994;10:264-9.
Grassi MP, Borella M, Clerici F, Perin C, Bini MT, Mangoni A. Reversible bilateral opercular syndrome secondary to AIDS-associated cerebral toxoplasmosis. Ital J Neurol Sci 1994;15:115-7.
Lang C, Reichwein J, Iro H, Treig T. Foix-Chavany-Marie-syndrome--neurological, neuropsychological, CT, MRI, and SPECT findings in a case progressive for more than 10 years. Eur Arch Psychiatry Neurol Sci 1989;239:188-93.
Mariani C, Spinnler H, Sterzi R, Vallar G. Bilateral perisylvian softenings: bilateral anterior opercular syndrome (Foix-Chavany-Marie syndrome). J Neurol 1980;223:269-84.
Mateos V, Salas-Puig J, Campos DM, Carrero V, Andermann F. Acquired bilateral opercular lesions or Foix-Chavany-Marie syndrome and eating epilepsy. J Neurol Neurosurg Psychiatry 1995;59:559-60.
Nisipeanu P, Rieder I, Blumen S, Korczyn AD. Pure congenital Foix-Chavany-Marie syndrome. Dev Med Child Neurol 1997;39:696-8.
Redondo L, Mir J, Perez de Leon JA, et al. HIV related Foix-Chavany-Marie syndrome. Neurologia 2003;18:741-5.
Sasaguri H, Sodeyama N, Maejima Y, Kanda T, Mizusawa H. Slowly progressive Foix-Chavany-Marie syndrome associated with chronic herpes simplex encephalitis. J Neurol Neurosurg Psychiatry 2002;73:203-4.
Shevell MI, Carmant L, Meagher-Villemure K. Developmental bilateral perisylvian dysplasia. Pediatr Neurol 1992;8:299-302.
Szabo K, Gass A, Rossmanith C, Hirsch JG, Hennerici MG. Diffusion- and perfusion-weighted MRI demonstrates synergistic lesions in acute ischemic Foix-Chavany-Marie syndrome. J Neurol 2002;249:1735-7. Erratum in: J Neurol 2003;250:380.
Villa G, Caltagirone C. Speech suppression without aphasia after bilateral perisylvian softenings (bilateral rolandic operculum damage). Ital J Neurol Sci 1984;5:77-83.
Weller M. Anterior opercular cortex lesions cause dissociated lower cranial nerve palsies and anarthria but no aphasia: Foix-Chavany-Marie syndrome and "automatic voluntary dissociation" revisited. J Neurol 1993;240:199-208.
Weller M, Poremba M, Dichgans J. Opercular syndrome without opercular lesions: Foix-Chavany-Marie syndrome in progressive supranuclear motor system degeneration. Eur Arch Psychiatry Neurol Sci 1990;239:370-2.
Guillain-Barre syndrome:Ropper AH. Further regional variants of acute immune polyneuropathy. Bifacial weakness or sixth nerve paresis with paresthesias, lumbar polyradiculopathy, and ataxia with pharyngeal-cervical-brachial weakness. Arch Neurol 1994;51:671-5.
Leukemia/lymphoma/carcinomatous meningitis:Ozcakar L, Akinci A, Ozgocmen S, Aksu S, Cetin E. Bell's palsy as an early manifestation of acute lymphoblastic leukemia. Ann Hematol 2003;82:124-6.
Sasaki MG, Leite PG, Leite AG, de Almeida SM. Bilateral peripheral facial palsy secondary to lymphoma in a patient with HIV/AIDS: a case report and literature review. Braz J Infect Dis 2002;6:50-4.
Schattner A, Kozack N, Sandler A, Shtalrid M. Facial diplegia as the presenting manifestation of acute lymphoblastic leukemia. Mt Sinai J Med 2001;68:406-9.
Lyme disease:Angerer M, Pfadenhauer K, Stohr M. Prognosis of facial palsy in Borrelia burgdorferi meningopolyradiculoneuritis. J Neurol 1993;240:319-21.
Christen HJ, Bartlau N, Hanefeld F, Eiffert H, Thomssen R. Peripheral facial palsy in childhood--Lyme borreliosis to be suspected unless proven otherwise. Acta Paediatr Scand 1990;79:1219-24.
Dotevall L, Hagberg L. Successful oral doxycycline treatment of Lyme disease-associated facial palsy and meningitis. Clin Infect Dis 1999;28:569-74.
Eng GD. Lyme disease presenting with bilateral facial nerve palsy. Arch Phys Med Rehabil 1990;71:749-50.
Graf M, Kristoferitsch W, Baumhackl U, Zeitlhofer J. Electrophysiologic findings in meningopolyneuritis of Garin-Bujadoux-Bannwarth. Zentralbl Bakteriol Mikrobiol Hyg [A] 1987;263:324-7.
Siwula JM, Mathieu G. Acute onset of facial nerve palsy associated with Lyme disease in a 6 year-old child. Pediatr Dent 2002;24:572-4.
Vanzieleghem B, Lemmerling M, Carton D, et al. Lyme disease in a child presenting with bilateral facial nerve palsy: MRI findings and review of the literature. Neuroradiology. 1998;40:739-42.
Mastoiditis/otitis media:Ochi K, Miyamoto Y, Ohashi T. Pediatric bilateral facial nerve palsy secondary to acute otitis media. Auris Nasus Larynx 2003;30 Suppl:S97-8.
Premachandra DJ, Radcliffe G. Bilateral facial nerve palsy following secretory otitis media. J Laryngol Otol 1989;103:685.
Tovi F, Leiberman A. Silent mastoiditis and bilateral simultaneous facial palsy. Int J Pediatr Otorhinolaryngol 1983;5:303-7.
Osteopetrosis:Bajaj S, Gupta SC, Nigam DK. Osteopetrosis with bilateral facial nerve palsy. J Assoc Physicians India 1986;34:529-30.
Benichou OD, Laredo JD, de Vernejoul MC. Type II autosomal dominant osteopetrosis (Albers-Schonberg disease): clinical and radiological manifestations in 42 patients. Bone 2000;26:87-93.
Sarcoidosis:George MK, Pahor AL. Sarcoidosis: a cause for bilateral facial palsy. Ear Nose Throat J 1991;70:492-3.
Glocker FX, Seifert C, Lucking CH. Facial palsy in Heerfordt's syndrome: electrophysiological localization of the lesion. Muscle Nerve 1999;22:1279-82.
James DG. Differential diagnosis of facial nerve palsy. Sarcoidosis Vasc Diffuse Lung Dis 1997;14:115-20.
Sugita M, Sano M, Uchigata M, Aruga T, Matsuoka R. Facial nerve enhancement on gadolinium-DTPA in a case with neurosarcoidosis. Intern Med 1997;36:825-8.
Vaphiades MS, Eggenberger E. Childhood sarcoidosis. J Neuroophthalmol 1998;18:99-101.
Vasculitis:Nikolaou AC, Vlachtsis KC, Daniilidis MA, Petridis DG, Daniilidis IC. Wegener's granulomatosis presenting with bilateral facial nerve palsy. Eur Arch Otorhinolaryngol 2001;258:198-202.
Stewart OG, Simmons IG, Martin MF, Morrell AJ. Bilateral facial nerve palsy associated with p-ANCA positive vasculitis in a patient with rheumatoid arthritis. Br J Ophthalmol 2001;85:1266-7.
Chitkara N, Bakshi N, Goyal N, Goyal P. Post-traumatic bilateral facial nerve palsy. J Otolaryngol 2002;31:192-3.
Gilchrist JM. AAEM case report #26: Seventh cranial neuropathy. Muscle Nerve 1993;16:447-52.
Gout O, Bonnaud I, Weill A, et al. Facial diplegia complicating a bilateral internal carotid artery dissection. Stroke 1999;30:681-6.
Iriarte J, Parra J, Kanner AM. Transient facial palsy in sphenoidal electrode placement. Epilepsia 1996;37:1239-41.
Lee FS, Chu FK, Tackley M, Wu AD, Atri A, Wessels MR. Human granulocytic ehrlichiosis presenting as facial diplegia in a 42-year-old woman. Clin Infect Dis 2000;31:1288-91.
Lee RT, Oster MW, Balmaceda C, Hesdorffer CS, Vahdat LT, Papadopoulos KP. Bilateral facial nerve palsy secondary to the administration of high-dose paclitaxel. Ann Oncol 1999;10:1245-7. Mallya KB, Mendis T, Guberman A. Bilateral facial paralysis following ethylene glycol ingestion. Can J Neurol Sci 1986;13:340-1.
Willis J, Atack EA, Kraag G. Relapsing polychondritis with multifocal neurological abnormalities. Can J Neurol Sci 1984;11:402-4.
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.
References:
Arias G, Nogues J, Manos M, Amilibia E, Dicenta M. Bilateral facial nerve palsy: four case reports. ORL J Otorhinolaryngol Relat Spec 1998;60:227-9.
Buyukavci M, Tan H, Akdag R. An alarming sign for serious diseases in children: bilateral facial paralysis. Pediatr Neurol 2002;27:312-3.
Keane JR. Bilateral seventh nerve palsy: analysis of 43 cases and review of the literature. Neurology 1994;44:1198-202.
Wormald PJ, Sellars SL, de Villiers JC. Bilateral facial nerve palsies: Groote Schuur Hospital experience. J Laryngol Otol 1991;105:625-7.
Bell palsy:Adour K, Wingerd J, Doty HE. Prevalence of concurrent diabetes mellitus and idiopathic facial paralysis (Bell's palsy). Diabetes 1975;24:449-51.
McGregor JA, Guberman A, Amer J, Goodlin R. Idiopathic facial nerve paralysis (Bell's palsy) in late pregnancy and the early puerperium. Obstet Gynecol 1987;69:435-8.
Price T, Fife DG. Bilateral simultaneous facial nerve palsy. J Laryngol Otol 2002;116:46-8.
Ralli G, Magliulo G. Bell's palsy and its recurrences. Arch Otorhinolaryngol 1988;244:387-90.
Sartoretti-Schefer S, Kollias S, Wichmann W, Valavanis A. T2-weighted three-dimensional fast spin-echo MR in inflammatory peripheral facial nerve palsy. AJNR Am J Neuroradiol 1998;19:491-5.
Shaikh ZA, Bakshi R, Wasay M, Dai A, Gosy E. Magnetic resonance imaging findings in bilateral Bell's palsy. J Neuroimaging 2000;10:223-5.
Sherwen PJ, Thong NC. Bilateral facial nerve palsy: a case study and literature review. J Otolaryngol 1987;16:28-33.
Stahl N, Ferit T. Recurrent bilateral peripheral facial palsy. J Laryngol Otol 1989;103:117-9.
Congenital:Baek SK, Chae SW, Jung HH. Congenital internal auditory canal stenosis. J Laryngol Otol 2003;117:784-7.
Bergstrom L, Baker BB. Syndromes associated with congenital facial paralysis. Otolaryngol Head Neck Surg 1981;89:336-42.
Carr MM, Ross DA, Zuker RM. Cranial nerve defects in congenital facial palsy. J Otolaryngol 1997;26:80-7.
Shah UK, Ohlms LA, Neault MW, et al. Otologic management in children with the CHARGE association. Int J Pediatr Otorhinolaryngol 1998;44:139-47.
Sjogreen L, Andersson-Norinder J, Jacobsson C. Development of speech, feeding, eating, and facial expression in Mobius sequence. Int J Pediatr Otorhinolaryngol 2001;60:197-204.
St Charles S, DiMario FJ Jr, Grunnet ML. Mobius sequence: further in vivo support for the subclavian artery supply disruption sequence. Am J Med Genet 1993;47:289-93.
Tellier AL, Cormier-Daire V, Abadie V, et al. CHARGE syndrome: report of 47 cases and review. Am J Med Genet 1998;76:402-9.
Verzijl HT, van der Zwaag B, Cruysberg JR, Padberg GW. Mobius syndrome redefined: a syndrome of rhombencephalic maldevelopment. Neurology 2003;61:327-33.
Foix-Chavany-Marie syndrome (Bi-opercular syndrome):Ann MY, Liu OK, Wu YL. Foix-Chavany-Marie syndrome. Zhonghua Yi Xue Za Zhi (Taipei) 2001;64:540-4.
Asenbauer B, McEntagart M, King MD, Gallagher P, Burke M, Farrell MA. Chronic active destructive herpes simplex encephalitis with recovery of viral DNA 12 years after disease onset. Neuropediatrics 1998;29:120-3.
Becker PS. Developmental Foix-Chavany-Marie syndrome: polymicrogyria or macrogyria? Ann Neurol 1990;27:693-4.
Becker PS, Dixon AM, Troncoso JC. Bilateral opercular polymicrogyria. Ann Neurol 1989;25:90-2.
Biller J, Asconape J, Challa VR, Toole JF, McLean WT. A case for cerebral thromboangiitis obliterans. Stroke 1981;12:686-9.
Broussolle E, Bakchine S, Tommasi M, et al. Slowly progressive anarthria with late anterior opercular syndrome: a variant form of frontal cortical atrophy syndromes. J Neurol Sci 1996;144:44-58.
Cellerini M, Mascalchi M, Salvi F, Muscas GC, Dal Pozzo G. MRI of congenital Foix-Chavany-Marie syndrome. Pediatr Radiol 1995;25:316-7.
Christen HJ, Hanefeld F, Kruse E, Imhauser S, Ernst JP, Finkenstaedt M. Foix-Chavany-Marie (anterior operculum) syndrome in childhood: a reappraisal ofWorster-Drought syndrome. Dev Med Child Neurol 2000;42:122-32.
Colamaria V, Sgro V, Caraballo R, et al. Status epilepticus in benign rolandic epilepsy manifesting as anterior operculum syndrome. Epilepsia 1991;32:329-34.
Duffau H, Karachi C, Gatignol P, Capelle L. Transient Foix-Chavany-Marie syndrome after surgical resection of a right insulo-opercular low-grade glioma: case report. Neurosurgery 2003;53:426-31.
Foix C, Chavany JA. Diplégie faciales (facio-linguo-pharingo-masticatrices), d'origine corticale, avec quelques considerations sur les paralysies pseudo-bulbaires et la localization des centres corticaux de l'estrémité céphalique. Ann Med 1926;20:480-98.
Foix C, Chavany JA, Marie J. Diplégie facio-linguo-masticatrice d'origine cortico-suous-cortical sans paralysie des members. Rev Neurol 1926;33:214-9.
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