Pediatrics & Neonatology
Volume 50, Issue 1 , Pages 36-38, February 2009

Möbius Syndrome as a Syndrome of Rhombencephalic Maldevelopment: A Case Report

  • Hsueh-Ting Huang

      Affiliations

    • Department of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan
    • ,
  • Chiao-Wen Hwang

      Affiliations

    • Department of Physical Medicine and Rehabilitation, Kaohsiung Veterans General Hospital, School of Medicine, National Yang-Ming University, Taipei, Taiwan
    • ,
  • Ping-Hong Lai

      Affiliations

    • Department of Radiology, Kaohsiung Veterans General Hospital, School of Medicine, National Yang-Ming University, Taipei, Taiwan
    • ,
  • Chu-Chin Chen

      Affiliations

    • Department of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan
    • Corresponding Author InformationCorresponding author. Department of Pediatrics, Kaohsiung Veterans General Hospital, 386 Ta-Chung 1st Road, Kaohsiung, Taiwan

Received 12 May 2008; received in revised form 15 October 2008; accepted 1 December 2008.

Article Outline

Möbius syndrome is a rare congenital disorder characterized by congenital facial weakness with impairment of ocular abduction. It is considered as a rhombencephalic disorder, and is often accompanied with hypoplasia of the pons and cerebellum. Here we report a male infant who had congenital facial asymmetry with absence of right-sided expression. Evident developmental delay was also found. The bilateral auditory brain stem response showed no response at 85 dB. A reconstructive brain magnetic resonance imaging (MRI) revealed the absence of the right facial nerve as well as hypoplasia of the pons and cerebellum of the same side. Some voluntary contraction over the patient's right lower face was noted after facial muscle electrical stimulation.

Key Words:  congenital facial palsy , developmental delay , Möbius syndrome , rhombencephalic disorder , unilateral involvement

No full text is available. To read the body of this article, please view the PDF online.

 

Back to Article Outline

References 

  1. Graefe von A . In:  Graefe von A ,  Saemisch T editor. Handbuch Der Gesammen Augenheilkunde . Leipzig: Engelmann; 1880;p. 60; [In German]
  2. Möbius PJ . Ueber angeborene doppelseitige Abducens-Facialis-Lähmung . Munch Med Wocheschr . 1888;35:91–94 08-11. [In German]
  3. Verzijl HT , van der Zwaag B , Lammens M , ten Donkelaar HJ , Padberg GW . The neuropathology of hereditary congenital facial palsy vs Möbius syndrome . Neurology . 2005;64:649–653
  4. Verzijl HT , van der Zwaag B , Cruysberg JR , Padlberg GW . Möbius syndrome redefined: a syndrome of rhombencephalic maldevelopment . Neurology . 2003;61:327–333
  5. Cattaneo L , Chierici E , Bianchi B , Sesenna E , Pavesi G . The localization of facial motor impairment in sporadic Möbius syndrome . Neurology . 2006;66:1907–1912
  6. Pedraza S , Gámez J , Rovira A , et al.   MRI findings in Möbius syndrome: correlation with clinical features . Neurology . 2000;55:1058–1060
  7. Verzijl HT , Padlberg GW , Zwarts MJ . The spectrum of Möbius syndrome: an electrophysiological study . Brain . 2005;128:1728–1736
  8. Donahue SP , Wenger SL , Steele MW , Gorin MB . Broad-spectrum Möbius syndrome associated with a 1;11 chromosome translocation . Ophthalmic Paediatr Genet . 1993;14:17–21
  9. Nishikawa M , Ichiyama T , Hayashi T , Furukawa S . Möbius-like syndrome associated with a 1;2 chromosome translocation . Clin Genet . 1997;51:122–123
  10. Slee JJ , Smart RD , Viljoen DL . Deletion of chromosome 13 in Moebius syndrome . J Med Genet . 1991;28:413–414
  11. Ziter FA , Wiser WC , Robinson A . Three-generation pedigree of a Möbius syndrome variant with chromosome translocation . Arch Neurol . 1977;34:437–442
  12. Kremer H , Kuyt LP , van den Helm B , et al.   Localization of a gene for Möbius syndrome to chromosome 3q by linkage analysis in a Dutch family . Hum Mol Genet . 1996;5:1367–1371
  13. Verzijl HT , van den Helm B , Veldman B , et al.   A second gene for autosomal dominant Möbius syndrome is localized to chromosome 10q, in a Dutch family . Am J Hum Genet . 1999;65:752–756

PII: S1875-9572(09)60028-0

doi:10.1016/S1875-9572(09)60028-0

Pediatrics & Neonatology
Volume 50, Issue 1 , Pages 36-38, February 2009

Article Tools