Historical note and nomenclature
Pathogenesis and pathophysiology
Prognosis and complications
Prognosis and complications
Brian H Le
Dr. Le has no relevant financial relationships to disclose.
Gerald Raymond (original author) and Javad Towfighi
September 7, 2007
Möbius syndrome: 352.6
Moebius syndrome: Q87.0
Moebius syndrome: %157900
Congenital facial diplegia syndrome; Congenital facial palsy syndrome; Congenital oculofacial paralysis; Congenital abducens-facial paralysis
Historical note and nomenclature
Mobius syndrome, congenital facial diplegia with restriction of lateral eye movements, was first described by von Graefe in 1880 (von Graefe 1880). Mobius, in his review of cranial nerve palsies in children, separated them into 6 groups where sixth and seventh nerve palsies occurring together were incorporated as one group (Mobius 1888; 1892). Henderson, in 1939, reviewed the clinical manifestations in 61 cases of congenital facial diplegia in which there were 45 cases of abducens palsies, 15 cases of external ophthalmoplegia, 6 cases with ptosis, 18 cases with involvement of the tongue, 19 cases with clubfeet, 13 cases with other brachial malformations, 8 cases with pectoralis muscle defects, and 6 cases with mental defect (Henderson 1939). Henderson's review incorporated all of the essential features of Mobius syndrome: (1) facial diplegia with other cranial nerve palsies, (2) malformations, particularly of the limbs, and (3) mental retardation with an incidence of 10%. Other clinical features have recently been described in association with Mobius syndrome, including upper labial deficiency (Sabbagh et al 2003), cataplexy (Tyagi and Harrington 2003), and poor impulse control with exhibitionism and aggression (Hedges et al 2003). The clustering of various craniofacial, musculoskeletal, and cardiac malformations as well as the mental retardation commonly observed in patients manifesting this syndrome has suggested that the entity exists along a heterogeneous spectrum. This gives rise to such terms as Mobius-like syndrome and Mobius sequence (Peleg et al 2001; Stromland et al 2002).
Mobius syndrome is characterized by congenital partial or complete facial diplegia often accompanied by other cranial nerve palsies and associated with other malformations of the limbs and orofacial structures. The facial palsy is typically noted in the newborn period due to difficulty with nursing and incomplete closure of the eyes while sleeping. Later, the inability to smile or the lack of movement while crying attracts concern. The facies are mask-like with inability to close the mouth and drooling of saliva, a troublesome symptom, especially in older individuals. Speech is indistinct, with difficulty making labial sounds. Recently, upper labial deficiency was recognized as a feature of Mobius syndrome that had previously been unreported (Sabbagh et al 2003).
The distribution of the facial palsy is often bilateral and incomplete (Henderson 1939). In Henderson's series, 19 of the 61 cases involved all 4 quadrants of the face, whereas 27 cases were partial (5 cases had insufficient information, and in the other 10 cases, no quadrant was completely paralyzed). In contrast to the usual distribution of nuclear or supranuclear lesions, the upper quadrants involving the frontalis muscles were predominantly affected.
Paralysis of the abducens nerve with inability to abduct the eye is frequently present and is often bilateral. The oculomotor nerve is occasionally involved. The hypoglossal nerve is commonly involved, with paralysis and hypoplasia of the associated musculature. Hypoglossal involvement is always accompanied by sixth nerve palsy (Henderson 1939; Kumar 1990). Other cranial nerves that have been affected include the trigeminal and those that supply the soft palate and pharynx.
Limb and craniofacial abnormalities are frequent. One third of children with Mobius syndrome have talipes equinovarus (Kumar 1990). Absence of the sternal head of the pectoralis muscle accompanied by hand malformations (Poland anomaly) is frequently seen. Other limb defects that have been reported include syndactylism, hypoplasia, and absence of digits. Craniofacial features have included epicanthus, external ear anomalies, bifid uvula, cleft palate, micrognathia, and, rarely, other branchial muscle defects (Domingos et al 2004). Global hypotonia, giving rise to symptoms suggestive of a severe infantile form of a congenital muscular disorder, may also be the presenting manifestation (Imamura et al 2007).
A classification and grading system has been proposed for Mobius syndrome to facilitate categorization and comparison of Mobius phenotypes for management as well as for studying outcome. Referred to as CLUFT, this system categorizes and grades the anatomic extent of cranial nerve deficits and musculoskeletal abnormalities, specifically evaluating the following: cranial nerves, lower limb, upper limb, face, and thorax (Abramson et al 1998). Recently, however, studies have defined and classified Mobius syndrome into two distinct neurophysiologic phenotypes. In one phenotype, patients have increased facial distal motor latencies (DML) and poor recruitment of small and polyphasic motor unit action potentials (MUAP). The second phenotype is characterized by normal facial distal motor latencies and neuropathic MUAP (Cattaneo et al 2006).
Aside from the neurologic and musculoskeletal features, numerous psychiatric and behavioral manifestations have been observed in association with Mobius syndrome (Briegel 2006); these include cataplexy (Tyagi and Harrington 2003). Additionally, a 20-year follow-up of a patient revealed poor impulse control, exhibitionism, and aggression (Hedges et al 2003). Although previous studies reported an increased prevalence of mental retardation, recent analyses demonstrated that the occurrence of mental retardation and memory dysfunction are not elevated relative to the clinically normal population (Verzijl et al 2005d). Recently, a case of Mobius syndrome with long-standing sleep disturbances, characterized by brief crying, vocalization, and twitching arm and leg movements during sleep, was described (Anderson et al 2007).
Recently, a case Wilm's tumor was described in a patient with Mobius syndrome (Yaris et al 2004). A case of Mobius syndrome with total anomalous pulmonary venous connection was recently characterized (Suvarna et al 2006), and a case with cavernous malformations was also described (Mut et al 2007).
No information was provided by the author.
The etiology of Mobius syndrome is multifactorial, implicating both genetic and environmental factors. Four genetic loci have been described: (1) the Mobius syndrome gene 1 on chromosome 13, (2) Mobius syndrome gene 2 on chromosome 3, (3) Mobius syndrome gene 3 on chromosome 10, and (4) Mobius syndrome gene 4 on chromosome 1 (Van der Zwaag et al 2002). Modes of inheritance have been postulated to range from autosomal recessive to autosomal dominant and X-linked. However, most cases have been sporadic (MacDermot et al 1990; Van der Zwaag et al 2002). Genetic studies have revealed various chromosomal abnormalities, mainly translocations, in association with Mobius syndrome (Hedges et al 2003; Kersey et al 2006).
Those cases that show brainstem injury are presumed to have been acquired in development and implicate destruction by ischemia or infections (Thakkar et al 1977; Towfighi et al 1979; Wilson et al 1982; Lipson et al 1990; Sherer and Spafford 1994; Sarnat 2004; Allen et al 2006). Several etiologies have been suggested from animal studies and case reports. These include the following: uterine artery clamping, attempted abortions with curettage at 12-weeks gestation (Lipson et al 1989), an unknown amount of methaqualone at 11-weeks gestation (Sudarshan and Goldie 1985), or with misoprostol, a synthetic prostaglandin E1 analog that is being used illicitly in Brazil and other countries as an abortifacient (Shepard 1995; Pastuszak et al 1998), ergotamine ingestion in early pregnancy (Smets et al 2004), hyperthermia in the first trimester (Superneau and Wertelecki 1985; Graham et al 1988; Lipson et al 1989); and maternal ingestion during the pregnancy of teratogenic agents such as thalidomide, alcohol, and cocaine (Elsahy 1973; Lipson et al 1989; Kankirawatana et al 1993; Puvabanditsin et al 2005).
Pathogenesis and pathophysiology
The pathogenesis of congenital facial diplegia is heterogeneous. Towfighi and colleagues divided the condition into 4 etiopathologic groups: (1) congenital hypoplasia of the cranial nerve nuclei, (2) primary peripheral nerve involvement, (3) necrosis of brainstem nuclei due to an anoxic-infectious cause, and (4) myopathies (Towfighi et al 1979).
In cases with hypoplasia or aplasia of the cranial nerve nuclei, there is an absence or diminution in the number of neurons in the various nuclei (Richter 1958; Towfighi et al 1979). The remaining neurons may be small or normal in size. Other brainstem structures such as the inferior olive may also be affected. Recent assessments have demonstrated, however, that even in patients demonstrating radiographic evidence of brainstem hypoplasia, the traversing motor and sensory tracts through the brainstem and the associated peripheral nerves may still be electrophysiologically intact (Verzijl et al 2005). Furthermore, recognizing the diverse pathogenetic spectrum of Mobius syndrome, recent studies have observed that this condition can be stratified into two neurophysiologic phenotypes: 1 with rhombencephalic maldevelopment with selective sparing of small-size motor units, and 1 likely related to an acquired injury during intrauterine life, giving rise to neurogenic remodeling of motor units (Cattaneo et al 2006).
Aplasia of the cranial nerve nuclei may result from loss of function of specific developmental genes. Mice with gene-targeted disruptions of the hoxb-2 locus demonstrated severe facial paralysis resulting from a failure to form the somatic motor component of the facial nerve (Barrow and Capecchi 1996). The mice also demonstrated sternal defects. At least a portion of this gene's effect may have resulted from the alteration in function of 2 other hox genes, hoxb-1 and hoxb-4.
Cases with apparent foci of necrosis in the brainstem show a decrease in neurons in the affected nuclei and calcifications or evidence of hemorrhage (Thakkar et al 1977; Towfighi et al 1979; Wilson et al 1982; Lipson et al 1990; Verzijl et al 2005c). These lesions are presumed acquired during fetal development and reflect ischemia of brainstem structures (Dooley et al 2004). This is supported by the observation that watershed (boundary zone) infarcts in the fetal and neonatal brainstem, as confirmed and dated by postmortem examination, can clinically express as multiple cranial neuropathies, failure of central respiratory drive, or apnea, as well as Mobius syndrome and the Pierre Robin sequence (Sarnat 2004). Additional support is similarly seen in associated anomalies such as the Poland anomaly and limb malformations, which are also presumed to have a vascular basis.
Peripheral neuropathy and myopathy are much less common manifestations and usually reflect more of an underlying generalized disorder involving either nerve or muscle. Included in this group is a disorder that has been reported as Mobius syndrome with peripheral neuropathy and hypogonadotrophic hypogonadism (Olson et al 1970; Abid et al 1978). This represents a distinct entity and should be separated from Mobius syndrome.
No information on incidence is available. However, more than 300 cases have been reported to date with no sex predilection (Gorlin et al 2001).
Acquired facial palsy from delivery may present bilaterally, but the distribution of the weakness and lack of other associated palsies usually allow differentiation. The cardiofacial syndrome with asymmetric crying facies, congenital heart defects, and other anomalies may be confused with Mobius syndrome. Other neuromuscular disorders including infantile spinal muscular atrophy, congenital forms of myasthenia gravis, myotonic dystrophy, facioscapulohumeral dystrophy, and other myopathies may present initially with facial weakness in the newborn period (Volpe 2001).
The oromandibular-limb hypogenesis disorders overlap with Mobius syndrome. All members of this group, including hypoglossia-hypodactylia, Hanhart syndrome, glossopalatine ankylosis, and Charlie M syndrome, have a variety of limb and craniofacial anomalies (Gorlin et al 2001).
As a syndrome with a heterogeneous spectrum of clinical manifestations and overlap, Mobius syndrome demonstrates marked similarities to the Athabascan brainstem dysgenesis syndrome, a newly described disorder characterized clinically by horizontal gaze palsy, sensorineural deafness, central hypoventilation, and developmental delay. Key features distinguishing this syndrome from Mobius syndrome are essentially the pattern of central nervous system findings, specifically sensorineural deafness and central hypoventilation (Holve et al 2003).
Diagnostic testing to evaluate the level of the lesion and pathogenesis is indicated. Because of the variable pathogenesis, there is no specific diagnostic testing for Mobius syndrome. EMG nerve conduction velocity should be performed to evaluate for other conditions in the differential diagnosis and to elucidate the level of the lesion. Results of such electrophysiological testing have previously demonstrated a spectrum of disturbance varying in degree of severity as well as neuroanatomic localization (Verzijl et al 2005a). Although head MRI and CT imaging are usually remarkable only for medial deviation of the eyes, other findings may include hypoplastic or dysplastic brainstem and bilateral symmetric calcifications adjacent to the fourth ventricle floor at the level of the sixth cranial nerve nuclei (Beerbower et al 1986; Kuhn et al 1990; Pedraza et al 2000; Dooley et al 2004). In a reported case series, imaging has also revealed absence of the facial nerve (Verzijl et al 2005b). Additionally, there has been a reported case in which the middle cerebellar peduncles were absent (Ouanounou et al 2005). Ophthalmological examination should be performed to evaluate the strabismus and to assist in management (Miller et al 1989). Overall, it appears that the most current and principle modality for diagnostic assessment and stratification of Mobius syndrome is electrophysiologic testing (Verzijl et al 2005; Cattaneo et al 2006).
Prognosis and complications
This is a static condition; however, mild improvement with age has been reported.
Complications will depend on degree of involvement but commonly include corneal irritation or ulceration, speech difficulties, aspiration, and feeding problems, particularly in the neonatal period resulting in poor growth. Speech difficulties and lack of facial expression may lead to the false impression of cognitive delay. Inability to express emotion may lead to social isolation (Terzis and Noah 2003).
When appropriate, management should include the following: correction of strabismus, protection of the cornea, attention to feeding and nutrition, speech-language therapy, and appropriate therapy and rehabilitation of any limb anomalies. Recent studies have documented the benefits of early dental management with orthodontic appliances, reporting improvement in palate expansion and decreased severity of micrognathia following 24 months of treatment and follow-up (Magalhaes et al 2006).
There have been several reconstructive procedures proposed for the management of the lack of facial animation. These generally involve the use of muscle transfers and multistage, microsurgical dynamic reconstruction involving nerve grafting with subsequent microneurovascular muscle transplant. Nevertheless, specific therapy and goals must be individualized (Zuker and Manktelow 1989; Terzis and Noah 2003).
Polyhydramnios secondary to impaired swallowing may complicate a pregnancy with an affected fetus (Sudarshan and Goldie 1985; Sherer and Spafford 1994). The limb abnormality may be detected on ultrasound (Brons et al 1990). In a retrospective review of a prenatal ultrasound of a child with Mobius syndrome, Yoon and colleagues were able to see hyperechoic signals in the brainstem consistent with calcifications (Yoon et al 1997).
The patient with Mobius syndrome may present several anesthetic problems including weak swallow, difficulty in maintaining secretions, and a history of aspiration resulting in chronic pulmonary changes. Use of antisialagogues--atropine, scopolamine, or glycopyrrolate--is recommended. The patient should also receive intensive respiratory support postoperatively. As the principle anesthetic concern centers on the propensity of patients with Mobius syndrome to encounter postoperative respiratory failure, consideration should be given to leaving the endotracheal tube in place to facilitate pulmonary care until it is certain that the patient is capable of handling secretions (Krajcirik et al 1985; Gondipalli and Tobias 2006).
Nonparalytic horizontal strabismus
acquired facial palsy from delivery
cardiofacial syndrome with asymmetric crying facies, congenital heart defects, and other anomalies
infantile spinal muscular atrophy
myasthenia gravis, congenital forms
oromandibular-limb hypogenesis disorders
Charlie M syndrome
Athabascan brainstem dysgenesis syndrome
For more specific demographic information, see the Epidemiology, Etiology, and Pathogenesis and pathophysiology sections of this clinical summary.
None selectively affected.
None selectively affected.
heredity may be a factor
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**References especially recommended by the author or editor for general reading.