eMedicine Specialties > Neurology > Pediatric Neurology

Mobius Syndrome: Treatment & Medication

Author: Cheryl Ann Palmer, MD, Professor, Departments of Pathology and Neurology, University of Alabama at Birmingham School of Medicine; Consulting Staff, Departments of Pathology and Neurology, University of Alabama at Birmingham Hospital; Consulting Staff, Departments of Pathology and Neurology, Veteran Affairs Medical Center; Consulting Staff, Department of Pathology, Children's Hospital of Alabama
Contributor Information and Disclosures

Updated: Mar 17, 2009

Treatment

Medical Care

Möbius syndrome is congenital and nonprogressive. However, no definitive treatment is available. To date, medical care is supportive and symptomatic.

  • Treatment for corneal ulcerations or abrasions may be required. This is secondary to exposure keratitis and conjunctivitis secondary to incomplete eyelid closure.
  • Congenital limb amputations or other musculoskeletal abnormalities may significantly compromise patients with Möbius syndrome. Splints, prostheses, or even prophylaxis for deep venous thrombosis may be indicated.
  • For a number of reasons, patients may be predisposed to infections.
    • Brainstem abnormalities may predispose patients to aspiration pneumonia, which may be complicated by pulmonary infections. Vigilance and a low threshold for treatment are required.
    • If the patient has structural anomalies of the ear, otitis media may complicate the patient's clinical course and require intervention.
  • Research into the psychological family dynamics of Möbius syndrome patients has drawn attention to the problems of parental bonding and possible disturbances of family care. This can occur because of a lack of feedback to the mother from the child, who may not be able to signal to her in the normal fashion by smiling or visual following.
  • When affected children reach an age of self-awareness, they realize that others notice the facial immobility. Facial mobility may increase with age. By school age, the condition may be less socially embarrassing than before. Family or individual counseling might prove helpful in these situations.
  • Neuropsychological and intelligence testing are helpful in predicting and assisting possible learning deficiencies, autism, or various visual apraxias.

Surgical Care

Surgical care is symptomatic or cosmetic but not curative of the underlying syndrome.

  • Clubfoot, frequently bilateral, occurs in almost one third of patients. In most, the deformity can be corrected partially or entirely by means of orthopedic procedures.
  • Airway functions commonly are compromised. Tracheotomy may be required to support the airway and permit tracheobronchial clearing.
  • With the diffuse incidence of feeding problems in children with Möbius syndrome, supplemental energy intake via a feeding gastrostomy tube may be required. Nissen fundoplication procedures have not been helpful in treating neurogenic dysphagia and associated aspiration.
  • Most ophthalmologists recommend delaying surgery for strabismus because the condition frequently improves with age. Ocular surgical procedures have been successful in some patients with Möbius syndrome. In older patients, insertion of a gold weight into the eyelid may allow lid closure to protect the cornea.
  • Reanimation procedures to counteract the facial nerve paralysis may be successful. Restoration of function may be more successful if surgery is performed before age 7 years.
    • Otolaryngologists and plastic surgeons have used the unaffected accessory nerve or the mesenteric branch of CN V as donors for reinnervation procedures.
    • Other surgeons have designed living devices, composed of muscle and tendon transplants combined with reinnervation procedures, that have restored at least partial control of the facial musculature.

Consultations

  • Associated deficiencies in Möbius syndrome and its variants require a multidisciplinary approach by skilled specialists.
  • Consultations may be required from pediatricians, general surgeons, pediatric dentists, orthopedic surgeons, ophthalmologists, pediatric otolaryngologists, psychologists, physiatrists, occupational and physical therapists, audiologists, and speech therapists9 .

Diet

If the brainstem is affected severely (enough to cause dysphagia), a dietician might assist with aspiration prevention.

Activity

Activity is not specifically limited in patients with Möbius syndrome, but it may be limited by the patient's physical abilities.

Medication

With few exceptions, pharmacologic intervention is used only for symptomatic treatment.

Antibiotics

Proper antibiotic therapy should be started in the event of infections such as pneumonia or otitis media.


Amoxicillin (Trimox, Amoxil, Biomox)

Ampicillin analog with broad-spectrum bactericidal activity against many gram-positive and gram-negative organisms.

Adult

250 mg PO tid for 10-14 d

Pediatric

<20 kg: 40 mg/kg/d PO divided q8h suggested for serious infections; for minor conditions, 20 mg/kg/d PO suggested
>20 kg: Administer as in adults

Reduces efficacy of oral contraceptives

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Periodically assess renal, hepatic, and hematopoietic functions; adjust dose in renal impairment; possible superinfection with mycotic or resistant bacterial pathogens


Trimethoprim and sulfamethoxazole (Bactrim)

Synthetic, broad-spectrum antibacterial combination. Inhibits bacterial synthesis of dihydrofolic acid by competing with PABA, inhibiting bacterial growth.

Adult

2 tab (each contains 400 mg SMZ and 80 mg TMP) PO q12h for 10-14 d

Pediatric

<2 months: Not recommended
Acute otitis media: 8 mg/kg TMP and 40 mg SMZ per 24 h, divided q12h

Warfarin may prolong PT; thiazide diuretics may increase incidence of thrombocytopenia in elderly patients; dapsone may increase levels of TMP and/or dapsone; may decrease hepatic clearance and half-life of phenytoin; can displace methotrexate from plasma protein-binding sites, increasing free concentrations; may increase hypoglycemic response of sulfonylureas; may decrease renal clearance of zidovudine, increasing zidovudine levels

Documented hypersensitivity; documented megaloblastic anemia due to folate deficiency; pregnancy at term; breastfeeding

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Discontinue at first appearance of rash or other adverse reaction; frequently obtain CBC count (if significantly reduced, discontinue therapy); diuresis, hypoglycemia, and goiter may occur; caution in folate deficiency (eg, alcoholism, elderly, patients receiving anticonvulsants, malabsorption syndromes); hemolysis may occur in G-6-PD deficiency; if signs of bone marrow depression occur, administer leucovorin (5-15 mg/d PO recommended) to restore normal hematopoiesis; because of unique immune dysfunction, patients with AIDS may not tolerate or respond to therapy; caution in renal or hepatic impairment (administer adequate fluid to prevent crystalluria and stone formation and monitor renal function during therapy)

More on Mobius Syndrome

Overview: Mobius Syndrome
Differential Diagnoses & Workup: Mobius Syndrome
Treatment & Medication: Mobius Syndrome
Follow-up: Mobius Syndrome
Multimedia: Mobius Syndrome
References
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References

  1. Von Graefe A, Saemisch T. Handbuch der gesammten Augenheilkunde. Leipzig, W Engelman. 1880;6:60.

  2. Mobius PJ. Uber angeboren doppelseitige abducens-facialis-lahmung. Munchen Medizinische Wochenschrift. 1888;35:91-4.

  3. Mobius PJ. Uber infantilen kernschwund. Munchen Medizinische Wochenschrift. 1892;39:17-21, 41-3, 55-8.

  4. Henderson JL. The congenital facial diplegia syndrome: clinical features, pathology and etiology. Brain. 1939;62:381-403.

  5. Verzijl HT, van der Zwaag B, Cruysberg JR, Padberg GW. Möbius syndrome redefined: a syndrome of rhombencephalic maldevelopment. Neurology. Aug 12 2003;61(3):327-33. [Medline].

  6. Baraitser M. Genetics of Möbius syndrome. J Med Genet. Dec 1977;14(6):415-7. [Medline].

  7. Strömland K, Sjögreen L, Miller M, Gillberg C, Wentz E, Johansson M, et al. Mobius sequence--a Swedish multidiscipline study. Eur J Paediatr Neurol. 2002;6(1):35-45. [Medline].

  8. Kumar D. Moebius syndrome. J Med Genet. Feb 1990;27(2):122-6. [Medline].

  9. Cohen SR, Thompson JW. Variants of Mobius' syndrome and central neurologic impairment. Lindeman procedure in children. Ann Otol Rhinol Laryngol. 1987;96(1 Pt 1):93-100. [Medline].

  10. Verzijl HT, van Es N, Berger HJ, Padberg GW, van Spaendonck KP. Cognitive evaluation in adult patients with Möbius syndrome. J Neurol. Feb 2005;252(2):202-7. [Medline].

  11. Gillberg C, Steffenburg S. Autistic behaviour in Moebius syndrome. Acta Paediatr Scand. Mar 1989;78(2):314-6. [Medline].

  12. Towfighi J, Marks K, Palmer E, Vannucci R. Möbius syndrome. Neuropathologic observations. Acta Neuropathol (Berl). Oct 1979;48(1):11-7. [Medline].

  13. Kanemoto N, Kanemoto K, Kamoda T, Hasegawa M, Arinami T. A case of Moebius syndrome presenting with congenital bilateral vocal cord paralysis. Eur J Pediatr. Aug 2007;166(8):831-3. [Medline].

  14. Griz S, Cabral M, Azevedo G, Ventura L. Audiologic results in patients with Moebiüs sequence. Int J Pediatr Otorhinolaryngol. Sep 2007;71(9):1457-63. [Medline].

  15. Sugarman GI, Stark HH. Mobius syndrome with Poland's anomaly. J Med Genet. 1973;10(2):192-6. [Medline].

  16. Online Mendelian Inheritance in Man. %157900 - MOEBIUS SYNDROME; MBS. OMIM - Online Mendelian Inheritance in Man. Available at http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=157900.

  17. Ziter FA, Wiser WC, Robinson A. Three-generation pedigree of a Möbius syndrome variant with chromosome translocation. Arch Neurol. Jul 1977;34(7):437-42. [Medline].

  18. Slee JJ, Smart RD, Viljoen DL. Deletion of chromosome 13 in Möbius syndrome. J Med Genet. Jun 1991;28(6):413-4. [Medline].

  19. Kremer H, Kuyt LP, van den Helm B, van Reen M, Leunissen JA, Hamel BC, et al. Localization of a gene for Möbius syndrome to chromosome 3q by linkage analysis in a Dutch family. Hum Mol Genet. Sep 1996;5(9):1367-71. [Medline].

  20. Nishikawa M, Ichiyama T, Hayashi T, Furukawa S. Mobius-like syndrome associated with a 1;2 chromosome translocation. Clin Genet. 1997;51(2):122-3. [Medline].

  21. Pastuszak AL, Schüler L, Speck-Martins CE, Coelho KE, Cordello SM, Vargas F, et al. Use of misoprostol during pregnancy and Möbius' syndrome in infants. N Engl J Med. Jun 25 1998;338(26):1881-5. [Medline].

  22. Puvabanditsin S, Garrow E, Augustin G, Titapiwatanakul R, Kuniyoshi KM. Poland-Möbius syndrome and cocaine abuse: a relook at vascular etiology. Pediatr Neurol. Apr 2005;32(4):285-7. [Medline].

  23. Singh B, Shahwan SA, Singh P, al-Deeb SM, Sharif H. Mobius syndrome with basal ganglia calcification. Acta Neurol Scand. Jun 1992;85(6):436-8. [Medline].

  24. Cattaneo L, Chierici E, Bianchi B, Sesenna E, Pavesi G. The localization of facial motor impairment in sporadic Möbius syndrome. Neurology. Jun 27 2006;66(12):1907-12. [Medline].

  25. Jaradeh S, D'Cruz O, Howard JF Jr, Haberkamp TJ, Konkol RJ. Mobius syndrome: electrophysiologic studies in seven cases. Muscle Nerve. Sep 1996;19(9):1148-53. [Medline].

  26. Briegel W. Neuropsychiatric findings of Mobius sequence -- a review. Clin Genet. Aug 2006;70(2):91-7. [Medline].

  27. Carr MM, Ross DA, Zuker RM. Cranial nerve defects in congenital facial palsy. J Otolaryngol. Apr 1997;26(2):80-7. [Medline].

  28. David TJ, Baumer JH. Problems of parental bonding and family care in child with the Möbius syndrome. J R Soc Med. Dec 1982;75(12):980-2. [Medline].

  29. Dooley JM, Stewart WA, Hayden JD, Therrien A. Brainstem calcification in Möbius syndrome. Pediatr Neurol. Jan 2004;30(1):39-41. [Medline].

  30. Johansson M, Wentz E, Fernell E, Strömland K, Miller MT, Gillberg C. Autistic spectrum disorders in Möbius sequence: a comprehensive study of 25 individuals. Dev Med Child Neurol. May 2001;43(5):338-45. [Medline].

  31. Kuhn MJ, Clark HB, Morales A, Shekar PC. Group III Mobius syndrome: CT and MR findings. AJNR Am J Neuroradiol. 1990;11(5):903-4. [Medline].

  32. Scarpelli AC, Vertchenko TB, Resende VL, Castilho LS, Paiva SM, Pordeus IA. Möbius syndrome: a case with oral involvement. Cleft Palate Craniofac J. May 2008;45(3):319-24. [Medline].

  33. Smets K, Zecic A, Willems J. Ergotamine as a possible cause of Möbius sequence: additional clinical observation. J Child Neurol. May 2004;19(5):398. [Medline].

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Further Reading

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Keywords

congenital facial diplegia, congenital nuclear agenesis, congenital nuclear hypoplasia, congenital oculofacial paralysis, Möbius syndrome, loss of function of motor cranial nerves, Poland anomaly, congenital facial paralysis, Poland sequence

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Contributor Information and Disclosures

Author

Cheryl Ann Palmer, MD, Professor, Departments of Pathology and Neurology, University of Alabama at Birmingham School of Medicine; Consulting Staff, Departments of Pathology and Neurology, University of Alabama at Birmingham Hospital; Consulting Staff, Departments of Pathology and Neurology, Veteran Affairs Medical Center; Consulting Staff, Department of Pathology, Children's Hospital of Alabama
Cheryl Ann Palmer, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuropathologists, Medical Association of the State of Alabama, Society for Neuro-Oncology, and Southern Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Robert Baumann, MD, Program Director, Professor, Departments of Neurology and Pediatrics, University of Kentucky
Robert Baumann, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American College of Epidemiology, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic
Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD, Assistant Professor, Department of Pediatrics, Division of Pediatric Neurology, Department of Neurology, Oregon Health and Science University; Consulting Staff, Shriners Hospital for Children
Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

 
 
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