Moebius's Syndroma—Congenital Oculofacial Paralysis

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Abstract
Congenital bilateral paralysis of the sixth and seventh cranial nerves constitutes an uncommon anomaly which may be associated with other abnormalities of development. The defect characteristically involves both external rectus muscles and the muscles of the face. In addition, there may be involvement of other muscles of the eye so that this disorder has been viewed as an intermediate condition between abduction paralysis and complete congenital external ophthalmoplegia. It has often been considered one of the congenital aplasiae involving motor cranial nerve nuclei. Two cases are presented. One is that of a 20-year-old girl with mental deficiency and seizures as well as bilateral paralysis of the sixth and seventh cranial nerves. The other is a 6-year-old boy who also exhibits multiple congenital deformities of the extremities, previously diagnosed as arthro-gryposis multiplex congenita. His sister, whose cranial nerves are entirely normal, also has congenital malformations of her extremities. Congenital oculofacial paralysis is easily diagnosed because of the appearance at birth, its non-progressive character and restricted site.

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