Mobius syndrome

Brian H Le MD (Dr. Le of Reading Hospital has no relevant financial relationships to disclose.)
Harvey B Sarnat MD FRCPC MS, editor. (Dr. Sarnat of the University of Calgary has no relevant financial relationships to disclose.)
Originally released December 28, 1993; last updated August 31, 2017; expires August 31, 2020

This article includes discussion of Mobius syndrome, congenital abducens-facial paralysis, congenital facial diplegia syndrome, congenital facial palsy syndrome, and congenital oculofacial paralysis. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

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. These features are observed in the newborn period, and modes of inheritance have been varied. In this update, the author incorporates recent clinical experiences with Mobius syndrome patients, including additional characteristics and syndromes associated with this entity.

Key points

 

• Mobius syndrome, which is characterized by congenital facial diplegia with cranial nerve palsies, exists as a heterogeneous clinical spectrum with many associated malformative lesions.

 

• To fulfill minimal diagnostic criteria for Mobius syndrome, there must be congenital, nonprogressive facial palsy accompanied by ocular abduction deficits; a newer classification scheme for Mobius syndrome is based on ocular motor phenotypes.

 

• Numerous behavioral manifestations have been observed in association with Mobius syndrome, including cataplexy, poor impulse control, and aggression.

 

• The etiology of Mobius syndrome is multifactorial, with both genetic implications (chromosomes 1, 2 10, 13) and intrauterine environmental factors (ischemia, infection); misoprostol use during pregnancy specifically conveys an increased risk to the fetus.

 

• Management strategies for Mobius syndrome rest with surgical correction of oral-facial abnormalities, with the objective of optimizing function. Timing of surgical intervention may play a crucial role in some patients. The possibility of vascular alterations such as facial artery agenesis in congenital facial palsy should be considered in preoperative planning. Management of Mobius syndrome is of interest to both medical and dental professionals, with advocacy for early rehabilitation to enhance recovery of function.

Historical note and terminology

Mobius syndrome, defined as 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, established 6 groups where sixth and seventh nerve palsies occurring together were incorporated as 1 group (Mobius 1888; Mobius 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 been described in association with Mobius syndrome, including facioscapulohumeral muscular dystrophy (Kolski et al 2008), 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), which are commonly encountered in the medical literature.

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