Möbius syndrome

Möbius syndrome is a rare congenital condition characterised by the absence or underdevelopment of the abducens nerve (CN VI) and facial nerve (CN VII) nuclei.

It is estimated that there is ~1 case per 100,000 births 4.

The earliest sign is the inability of the newborn to suckle, with an expressionless face, floppy limbs and drooling.

Aetiology is multifactorial, and several theories have been proposed, with the most supported theory being a of transient ischaemic or hypoxic insult to the fetus. Infectious and genetic aetiologies have also been proposed.

Additional brainstem involvement is also common and may include other cranial nerves as well as the musculoskeletal system. Grading schema have been proposed which include these other manifestations (e.g. CLUFT (cranial nerve, lower limb, upper limb, face, and thorax)).

Associations

The combination of Möbius syndrome and Poland syndrome has been well described (thus Poland-Möbius syndrome) 3.

MRI brain

MRI findings are those of brainstem hypoplasia (pons and medulla) with straightening of the floor of the fourth ventricle as a result of absence of the facial colliculus. Cerebellar hypoplasia, calcification of the pons in the region of the abducens nuclei and even absence of the middle cerebellar peduncles have been reported.

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The clinical syndrome was first described by Paul Julius Möbius (1853-1907): German neurologist.

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Article Information

rID: 1711
Section: Syndromes
Synonyms or Alternate Spellings:
  • Mobius syndrome
  • Moebius syndrome
  • Moebius sequence
  • Mobius sequence
  • Sindrome de Moebius

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