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Genetic and Rare Diseases Information Center (GARD)

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Brown-Vialetto-Van Laere syndrome


Other Names for this Disease
  • Pontobulbar palsy and neurosensory deafness
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Overview


Brown-Vialetto-Van Laere syndrome (BVVL) is a type of degenerative nerve disease. Initial symptoms occur in infancy to early adulthood and progress with time. The rate of progression can vary from person to person. The syndrome is generally characterized by sensorineural deafness, paralysis of the cranial nerves, and lower and upper motor neuron disease. Signs and symptoms may include vocal cord paralysis, drooping eyelids, facial weakness, slurred speech, difficulty swallowing, diminishing eye sight, neck and shoulder weakness, weakness in the arms and legs, autonomic dysfunction, and difficulty breathing. The cause of the syndrome is currently unknown and treatment is supportive. Both genetic and sporadic cases have been reported in the medical literature.[1]
Last updated: 3/23/2010

References

  1. Sathasivam S. Brown-Vialetto-Van Laere syndrome. Orphanet J Rare Dis. 2008;
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Basic Information

  • Genetics Home Reference (GHR) contains information on Brown-Vialetto-Van Laere syndrome. This website is maintained by the National Library of Medicine.

In Depth Information

  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Brown-Vialetto-Van Laere syndrome. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles