Summary
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.
Zusammenfassung
Es werden die klinisch-pathologischen Befunde angeführt, die bei einem jungen Patienten mit Diplegia-oculo-facialis-congenita-Parese der Pharynx- und Larynxmuskulatur, bei gleichzeitiger Tetraparese, beobachtet wurden.
Zu den erwähnten Symptomen und Syndromen kommt ein komplexes pathologisches Bild von Mißbildungen hinzu wie: das Fehlen der Bulbi olfactorii, Anomalien des Hippocampus, Hypoplysis des Corpus callosum und des Cerebellum und eine hochgradige Hypoplasie der efferenten Cortexbahnen.
Die Hypoplasie der efferenten Cortexbahnen und die dadurch bedingte Denervation der Hirnnervenkerne wird als die Ursache des Möbius-ähnlichen Syndroms angesehen.
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Nardelli, E., Vio, M., Ghersini, L. et al. Möbius-like syndrome due to multiple cerebral abnormalities including hypoplasia of the descending tracts. J Neurol 227, 11–19 (1982). https://doi.org/10.1007/BF00313542
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DOI: https://doi.org/10.1007/BF00313542