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Purpose: Despite its clinical implications, the MRI features of developmental facial paresis (DFP) were described in a few case reports. This study aims to describe MRI features of DFP in relation to the embryological development with a proposed radiological new grading system., Methods: The clinical records and MRI of the brain and internal auditory canal of 11 children with DFP were retrospectively reviewed. The following sequences were analyzed: axial, oblique sagittal SPACE of the internal auditory canal and brainstem; axial T2, T1WI and coronal T2WI of the brain. The severity of the maldevelopment of the seventh nerve was graded from 0 to 4: 0 = no abnormalities, 1 = unilateral facial nerve hypoplasia, 2 = unilateral facial nerve aplasia, 3 = aplasia or hypoplasia involving facial nerves on both sides, and 4 = facial nerve aplasia or hypoplasia associated with other cranial nerve palsy., Results: Isolated facial nerve palsy was diagnosed in seven patients. It was of grade 1 in five and grade 3 in two. Hypoplasia of the nerve with interrupted course was encountered in two cases. Other associated cranial nerve abnormalities (grade 4) were seen in four patients; two of them were diagnosed previously as Moebius syndrome. In addition to inner ear anomalies, middle and external ear and parotid gland anomalies were described., Conclusion: To our knowledge, this is the largest series of patients with DFP that represents a continuum of isolated and combined malformations. Understanding of embryological basis can give insights into the anomalous development of the facial nerve.

Congenital cranial dysinnervation disorders (CCDDs) are congenital non-progressive, sporadic or familial abnormalities of cranial musculature that result from developmental abnormalities of, or the complete absence of, one or more cranial nerves with primary or secondary muscle dysinnervation. These disorders include vertical disorders of ocular motility, horizontal disorders of ocular motility and disorders with abnormalities of facial motility that were previously referred to as "congenital fibrosis syndromes". The advances in clinical and molecular genetics of congenital cranial dysinnervation disorders are reviewed.

Two brothers born to a healthy, consanguineous Spanish couple have a syndrome of Möbius sequence with involvement of cranial nerves V, VI, VII, IX, and XII, central nervous system malformations; characteristic face with creased earlobes, short philthrum, and a short, arched upper lip, skeletal anomalies with short sternum and delayed bone maturation, hypogenitalism, and profound mental retardation. We suggest that this is a new multiple congenital anomalies condition and mental retardation (MCA/MR) syndrome with autosomic recessive inheritance., (Copyright 1999 Wiley-Liss, Inc.)

Methods: Cranial nerve defects were enumerated in 29 children with congenital facial palsy presenting for reanimation via chart review with a view to determining unaffected donor nerves. The literature was searched via Medline to reveal 186 additional case reports that were complete enough in their description to allow assessment of cranial nerve defects., Results: The group presenting for reanimation comprised 24 females and 5 males. All of the males had unilateral isolated facial nerve paralysis. Of the females, 9 had isolated unilateral palsy and 14 had bilateral palsy, and half of these were isolated. Six had bilateral abducens nerve palsy, fulfilling the original definition of Möbius syndrome. Of these, 3 had a right-sided hypoglossal weakness, one had a bilateral oculomotor palsy, and one had a bilateral trochlear palsy. One girl had bilateral facial and auditory involvement. The literature review revealed a group that was 60% male, with 85% having bilateral facial palsy. Abducens palsy was present in 68%, glossopharyngeal in 28%, hypoglossal in 26%, and oculomotor in 20%. The group was very heterogeneous in many ways., Conclusions: There are many theories of pathogenesis of this syndrome. Isolated facial palsy is in concordance with previous findings of nerve lesions within the temporal bone. Multiple cranial nerve defects are more likely to have brainstem lesions, related to prenatal ischemic events. The cranial nerve least likely to be involved was the accessory nerve, suggesting that this may be a reliable donor for reanimation procedures.

A child with Möbius syndrome (facial diplegia) was studied during affective interplay with both parents at six and 10 months, then re-assessed with the Ainsworth Strange Situation at 14 months. The child demonstrated normal cognitive development, but insecure-resistant attachment. This experiment of nature is presented as an example of resilience in the field of social communication of affect and discussed as comparable to examples found in the fields of motor co-ordination and expressive language.