Programm                 "Degeneration und Regeneration– Grundlagen, Diagnostik und Therapie"

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The Oculofacial Paresis (Moebius Syndrome)

Weich C. M., Käsmann-Kellner B., Kootz D., Löw U., Ruprecht K. W.
Universitäts-Augenklinik, Homburg/Saar

Purpose: The Moebius syndrome (OMIM 157900, Chr. 13q12) is characterized by an inborn disturbance of the horizontal eye movements in combination with variable paresis of the facial nerve. In most cases facial paresis is symmetrical on both sides leading to the pathognomical puppet-like face and to open eyes even in crying babies. Due to the aplasia of the abducens nucleus there is an abducens paralysis as well as horizontal gaze paresis. Since vertical eye-movements are intact, this syndrome leads to the clinical picture bilateral abducent nerve paralysis with crossed-eye convergence. The Moebius-syndrome occurs sporadically, nevertheless familial aggregations are reported.
Method: Five patients with eye-movement disturbances, and variable intensity of facial paralysis have been presented in our clinic. Two of them (n = 2) were siblings, who showed on inspection an orthophoria, all eye movements, however, were completely defect resembling a complete ophthalmoplegia. The examinations of other three patients showed bilateral abducens paralysis. Due to this combination we could establish the diagnosis of Moebius-syndrome in these patients. The patients not only showed a variable degree of Moebius-syndrome but also different combinations of associated malformations. In an infant aged one month there were additionally bilateral ptoses, additional Poland-syndrome, severe hearing loss and a cranio-facial asymmetry with retrognatia. Th

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