Möbius-like syndrome due to multiple cerebral abnormalities including hypoplasia of the descending tracts. A case report.
The clinicopathological findings are reported in a child with congenital oculofacial diplegia, paresis of pharyngeal and laryngeal muscles, and concomitant tetraparesis. The constellation of signs and symptoms correlates with a complex picture of maldevelopment, including absence of olfactory bulbs, hippocampal abnormalities, hypoplasia of the corpus callosum and of the cerebellum, and severe hypoplysia of the descending pathways. We stress the role played by the hypoplasia of the descending fibres, with consequent denervation of the cranial nerve nuclei, in producing the Möbius-like picture.
D000015:Abnormalities, Multiple / Q000473:pathology*; D001921:Brain / Q000002:abnormalities*; D003391:Cranial Nerves / Q000002:abnormalities; D005145:Face / Q000002:abnormalities*; D006801:Humans; D007223:Infant; D008297:Male; D010291:Paresis / Q000151:congenital; D011712:Pyramidal Tracts / Q000002:abnormalities