Möbius' syndrome II

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A very rare syndrome characterized by congenital palsy of the external rectus and facial muscles, usually bilateral, associated with paralysis of the sixth and seventh nerves.

Description

In this very rare syndrome, the abducens and facial nerves, which originate in the brain stem, may not develop from birth. It is characterized by congenital palsy of the external rectus and facial muscles, usually bilateral, associated with paralysis of the sixth and seventh nerves. The patient has a masklike expression and is unable to abduct the eyes beyond midpoint. Involvement of the jaw may cause feeding problems.

Other central nervous system dysfunctions may cause abnormalities in the hands, hips, and feet. The clinical features are not constant. Mental retardation occurs in approximately ten percent of cases. The condition has been observed in siblings and is thought to be genetic.

The disorder may have a number of different causes, one author ascribes it to aplasia of the nuclei of the abducens and facial nerves. According to one author it was first described by von Graefe in 1880. However, ince Albrecht von Graefe died in 1870, this probably refers to the textbook by Alfred Graefe and Edwin Theodor Saemisch. It was described by Möbius in 1888 and again in 1892 when he reported 44 cases and applied the term «nuclear atrophy.»

Bibliography

  • P. J. Möbius:
    Über angeborene doppelseitige Abducens-facialislähmung.
    Münchener medizinische Wochenshrift, 1888, 6: 108-111.
    Über infantilen Kernschwund.
    Münchener medizinische Wochenshrift, 1892, 39: 17.
  • Alfred Graefe and Edwin Theodor Saemisch:
    Handbuch der gesammten Augenheilkunde.
    Volume 6, page 60. Leipzig, Engelmann, 1880.

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