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Mobius Syndrome

Mobius syndrome* is a rare genetic disorder caused by the absence or underdevelopment of the sixth and seventh cranial nerves. Individuals with Möbius syndrome manifest varying degrees of facial paralysis which may affect eye movement, facial expression, and oral function.

Characteristics of Möbius syndrome include an inability to suck and excessive drooling, leading to problems with feeding, swallowing, and aspiration. Hypotonia of the upper body may result in scoliosis. Motor delays and deformities of the tongue, jaw, and limbs may also occur. Approximately 10 percent of affected individuals experience developmental delays.

Airway clearance indications which may be present in Möbius syndrome include:

• Abnormalities in oral-motor function and coordination that can result in swallowing difficulties, leading to choking and aspiration. Poor cognitive function may exacerbate these problems.
• Inadequate control of respiratory muscles due to hypotonia can impair the normal cough reflex and, as a result, interfere with the ability to clear aspirated material and pulmonary secretions from the airways.
• Retained secretions provide a cultural medium for bacterial pathogens and can initiate a vicious cycle of mucus obstruction, recurrent respiratory infection, and progressive pulmonary compromise.
• Restrictive lung disease, characterized by static or diminished lung volumes and vital capacities, can result from deformities of the spine and thorax. The ability to take a big breath, to generate expiratory force, and to cough effectively are typically affected.

Aggressive bronchial hygiene, including daily Airway Clearance Therapy, is an essential component in the management of patients with pulmonary complications from Möbius syndrome. Conscientious attention to daily secretion clearance may prevent or minimize the pulmonary complications associated with this disorder.

* Variations: Moebius syndrome; Mobius syndrome

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Sources

Thoene JG, Coker NP. Moebius syndrome. In: Thoene JG, Coker NP, eds. Physicians' Guide to Rare Diseases, 2^nd ed. Montvale, NJ: Dowden Publishing; 1995:106. National Institute of Neurological Disorder and Stroke. Mobius syndrome fact sheet. Available at:http://www.ninds.nih.gov/patients/disorders/mobius/mobius.html. Accessed March 23, 2000.

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