• Möbius syndrome is defined as congenital facial weakness combined with abnormal ocular abduction. (medscape.com)
  • Möbius syndrome is congenital and nonprogressive. (medscape.com)
  • Von Graefe and Möbius accepted only cases with congenital facial diplegia and bilateral abducens nerve palsies as constituting Möbius syndrome. (medscape.com)
  • These investigators require the presence of a congenital musculoskeletal anomaly in order to make the diagnosis. (medscape.com)
  • A striking feature in persons with Möbius syndrome is the high incidence of associated congenital deformities. (medscape.com)
  • A congenital condition called the Poland sequence, characterized by ipsilateral hand malformations and by partial or complete absence of the pectoralis muscles and breast, is concurrent with Möbius syndrome in approximately 15% of patients. (medscape.com)
  • It often involves abnormalities of the extremities, including absence of the pectoralis major muscle in Poland syndrome. (medscape.com)
  • An example of a development cause is Möbius syndrome, which has an incidence of 1 per 50,000 births. (medscape.com)
  • Numerous theories exist concerning the primary underlying pathogenesis of Möbius syndrome. (medscape.com)
  • Developmental causes The causes of developmental facial paralysis are numerous and may be associated with syndromes and teratogens. (medscape.com)
  • A neurophysiologic study of patients with sporadic Mobius syndrome demonstrated 2 distinct groups characterized by 1) increased facial distal motor latencies (DML) and poor recruitment of small neuropathic motor unit action potentials (MAUP) and 2) normal facial DMLs and neuropathic MAUPs. (medscape.com)
  • In 1979, Towfighi et al proposed a classification system for Möbius syndrome based on pathologic differences observed in studies of patients with the syndrome. (medscape.com)
  • A few families with Möbius syndrome have been described, but most cases are sporadic. (medscape.com)
  • Brainstem abnormalities can predispose patients to aspiration pneumonia, which may be complicated by pulmonary infections. (medscape.com)
  • In patients with severe brainstem compromise that causes dysphagia, aspiration, and an inability to protect the airway, death may occur at a young age. (medscape.com)
  • If the brainstem is affected severely (enough to cause dysphagia), a dietician may assist with aspiration prevention. (medscape.com)
  • Whether nerve, brainstem, or muscle aplasia is the primary event has not been established. (medscape.com)
  • Recognition of the fact that affected regions have no functional neuromuscular system explains why nerve grafting, cross-face grafting, and cranial nerve (CN) substitution techniques have had poor success in animating the faces of patients with Möbius syndrome. (medscape.com)
  • If the paralysis does not resolve, it may affect the child's future speech, expressions of emotion, and mastication. (medscape.com)
  • Complications of Möbius syndrome depend on the severity of the patient's deficits. (medscape.com)
  • If the patient has structural anomalies of the ear, otitis media may complicate the patient's clinical course and require intervention. (medscape.com)
  • Activity is not specifically limited in patients with Möbius syndrome, but it may be limited by the patient's physical abilities. (medscape.com)
  • [ 7 ] In a nationwide Dutch survey reported in 2003, the prevalence of Möbius syndrome was at least 0.002% of births (4 cases per 189,000 newborns) for the years 1996-1998. (medscape.com)
  • Autistic disorder is the most severe form of autism spectrum disorders (ASDs), which include Asperger's syndrome and pervasive developmental disorders (PDDs) not otherwise specified. (nih.gov)
  • Research into the psychological family dynamics of Möbius syndrome patients has drawn attention to the problems of parental bonding and possible disturbances of family dynamics. (medscape.com)