Mobius Syndrome Due to Brain Stem Tegmental Necrosis

Abstract

Möbius syndrome undoubtedly results from a variety of disorders affecting central or peripheral portions of appropriate cranial nerves or their target muscles. Pathological alterations observed in cranial nuclei are most often viewed as aplastic or dysplastic lesions. Two patients with Möbius syndrome with associated facial and skeletal malformations showed mineralized necrotic foci in multiple brain stem nuclei. Prenatal encephalomalacic lesions represent the pathological basis for some cases of congenital static Möbius syndrome.

Limb deficiency with or without Möbius sequence in seven Brazilian children associated with misoprostol use in the first trimester of pregnancy

“…Hyperthermia during early pregnancy was reported by Graham et al [1988]. Benzodiazepine ingestion by the mother was noted by Courtens et al [19921. Some cases of Miibius sequence must result from prenatal brainstem ischemia with necrosis and occasional calcification of the facial nuclei [Thakkar et al, 1977;Govaert et al, 19891.…”

Möbius sequence, Robin complex, and hypotonia: Severe expression of brainstem disruption spectrum versus Carey–Fineman–Ziter syndrome

“…The development of MS and limb defects after misoprostol exposure illustrates this vascular theory. The presence of calcifications in the floor of the 4th ventricle, observed in Patients 1, 2, and 4, and in some literature cases [Richter, 1958;Thakkar et al, 1977;Bouwes, Bavinck, and Weaver, 1986] may be a clue to this disruptive mechanism. An interesting patient with Poland-Möbius syndrome and central hypoventilation was reported with brainstem atrophy and calcifications visible on MRI [Fujita et al, 1991].…”

A severe case of Moebius syndrome with calcification on the fourth ventricular floor

“…The microcalcification on the fourth ventricular floor was explained as a consequence of damage to the tegmentum of the mid brain that was secondary to a blood flow disruption. The ischemic process may result in gliosis/calcification in and around the cranial nerve nuclei in the tegmentum (Thakkar et al 1977).…”

“…However, some patients with the syndrome die within one year of life, mainly due to respiratory insufficiency caused by aspiration pneumonias (Thakkar et al 1977). The clinical severity will be dependent on the degree of the brain stem damage.…”