Möbius and Möbius-like patients: etiology, diagnosis, and treatment options

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History

The unusual combination of developmental facial paralysis and restriction of horizontal eye movement was first described by v. Graefe in 1880 [1].

The German neurologist Paul Julius Möbius was the first to compare his own cases with case reports from Chrisholm [2] and Haarlan [3]. He suggested that the bilateral paralysis of the 7th and 6th cranial nerves might be an independent pathological entity with the presumptive term the infantile Kernschwund [4], [5]. His name was later used as an eponym.

Clinical findings

The paralysis of the orbicularis oculi muscle is the first sign of Mobius' syndrome and can be observed soon after birth; however, the condition is unfamiliar within the pediatric specialty, so diagnosis is made in later ages. Upon further development it becomes noticeable that the child does not smile or show other features of nonverbal communication. The mask-like faces are the dominant clinical finding associated with drooling and indistinct speech. If the facial paralysis is incomplete, the

Differential diagnosis

Physicians should consider the following possible differential diagnoses: glossopalatine-ankylosis syndrome, hypoglossal-hypodactyly syndrome, and Charlie-M syndrome [23]. In addition, there is a growing list of unrelated conditions with bilateral facial diplegia that must be considered [42], [43]. The combination of multiple cranial nerve deficiencies is also found in patients with CHARGE association [44].

Epidemiology

Facial paralysis is uncommon in newborns; the incidence rate has been reported to be between 0.2% and 6.9% [45]. Möbius' syndrome is very rare; in the past 120 years, approximately 320 cases have been reported in the literature. Most of these are smaller cohorts focusing on exceptional findings. The incidence in the population has not been determined yet [34]. The majority of cases occur sporadically, but undiagnosed cases are highly possible. Despite the apparent clinical features, the

Classification

Möbius' syndrome presents heterogeneously. The great variety of clinical findings dictates the need for a classification system. A clinical classification by Tentamy and McKusik [22] describes three groups: (1) isolated VI and VII cranial nerve palsy; (2) VI and VII cranial nerve palsy in association with arthrogryposis multiplex congenita; and (3) VI and VII cranial nerve palsies with associated limb anomalies. Based on neuropathological observations in one case and evaluation of 14 additional

Etiology

Because of the variety of clinical findings the etiology of Mobius' syndrome is still unknown. It remains unknown whether the developmental mishap is dysplastic or degenerative. Pathological findings differ between a primary ectodermal dysplasia of the midbrain with secondary mesodermal defect [50], [51] and a primary mesodermal defect [52], [53]. Besides myopathy [53], findings have pointed toward a peripheral neuropathy [54]. MRI, CT scans, and autopsy materials have shown calcification in

Treatment

Surgical treatment of Möbius patients focuses mainly on correction of limb and ocular deficiencies (eg, talipes correction and strabismus surgery). The inability of Möbius patients to express happiness, sadness, or anger through nonverbal communication can result in severe introversion and reclusive personality [82], [83], [84]. Because of their expressionless appearance and speech impairment, these patients are often considered to be mentally retarded [85], [86].

The restoration of even a small

Summary

The surgical goal in Möbius patients is far more modest and differs from patients with unilateral developmental facial paralysis. It is impossible to restore a true smile in these mask-like, expressionless faces. Despite sophisticated microneurovascular transplantations, movement can only be restored along one vector and enhanced firmness in the cheeks, thus multiple differentiated facial animation is not achievable.

A detailed neurological evaluation can identify possible motor donors or

Acknowledgements

The authors express their gratitude to Diane Mocion for providing medical illustrations and David Beck for photographs.

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