PubMed HealthSearch

PubMed · 506685

Möbius syndrome. Neuropathologic observations.

Abstract

Neuropathologic findings in an infant with congenital right facial and bilateral lateral rectus palsy (Möbius syndrome) are presented. Multiple microscopic foci of necrosis were found in the lower pontine tegmentum, involving the region of the intramedullary course of 6th and 7th cranial nerves. The significance of pathologic findings in this infant and 14 additional published cases of Möbius syndrome is discussed.

Explore related subjects

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

J Towfighi, K Marks, E Palmer, R Vannucci. 1979. Möbius syndrome. Neuropathologic observations.. https://doi.org/10.1007/bf00691785

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

Acquired oculomotor, trochlear, and abducent cranial nerve palsies in pediatric patients.

Between January 1966 and December 1988, 160 pediatric patients (age range, 0 to 17 years) were seen at the Mayo Clinic with an acquired oculomotor (35 patients), trochlear (19 patients), abducent (88 patients), or multiple (18 patients) cranial nerve palsy. The clinical findings in the 160 pediatric patients were compared with the results obtained in other reviews of cranial nerve palsies in the pediatric age group and with the adult Mayo Clinic patients with acquired cranial nerve palsies. Trauma was the most common reason for an acquired cranial nerve palsy in our pediatric group. The percentage of patients with an acquired cranial nerve palsy resulting from trauma was significantly greater in the pediatric group (42.5%) than in adults (15.4%) (P < .01). The difference between the percentage of adults (15.2%) and pediatric patients (16.9%) with a cranial nerve palsy secondary to a neoplasm was not statistically significant (P = .28).

Abducens Nerve

Distinct populations of neurons in the ventromedial periaqueductal gray project to the rostral ventral medulla and abducens nucleus.

Following microinjections of a colloidal gold complex into the nucleus paragigantocellularis (PGi) of the ventral medulla, and of latex microspheres into the nucleus abducens (Abd) of the same animal, retrogradely labeled neurons were identified in the region of the contralateral supraoculomotor nucleus (SOM) in the ventromedial periaqueductal gray (PAG). Neurons labeled from the Abd were found in the SOM in the ventromedial PAG throughout the midbrain, as well as scattered ventrally in the oculomotor nucleus. Neurons in the SOM area retrogradely labeled from the PGi were most numerous rostral to the dorsal raphe nucleus and extended throughout the level of the oculomotor nucleus. Direct comparison of the two labels revealed that the neurons that project to the Abd were located slightly more ventrally than PGi-projecting neurons. Almost no doubly labeled neurons were identified, although singly labeled neurons formed adjacent but separate populations. These results indicate that neurons in the SOM area projecting to the PGi are distinct from those projecting to the Abd, and that the PGi-projecting neurons are probably not pre-oculomotor neurons. Given their more dorsal location and the nature of their target neurons in the rostral ventrolateral medulla, PGi-projecting neurons may be related to visceromotor, as opposed to oculomotor functions.

Abducens Nerve