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Diseases

Genetic and Rare Diseases Information Center (GARD)

Monoamine oxidase A deficiency


Other Names for this Disease
  • Brunner syndrome
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Overview

Monoamine oxidase A deficiency is a rare condition that is characterized by mild intellectual disability and behavioral difficulties (including aggressive and sometimes violent behaviors and autistic features). Affected people may also experience night terrors, tremor, stereotypical hand movements, and/or occasional body twitches. Signs and symptoms generally develop in childhood and the condition is seen almost exclusively in males.[1][2] Monoamine oxidase A deficiency is caused by changes (mutations) in the MAOA gene and is inherited in an X-linked recessive manner.[3] Treatment is based on the signs and symptoms present in each person. Some recent studies suggest that cautious treatment with certain medications (called selective serotonin reuptake inhibitors) and dietary modifications can improve symptoms.[2][4]
Last updated: 3/30/2016

References

  1. Monoamine oxidase A deficiency. Orphanet. February 2012; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=3057.
  2. Palmer EE, Leffler M, Rogers C, Shaw M, Carroll R, Earl J, Cheung NW, Champion B, Hu H, Haas SA, Kalscheuer VM, Gecz J, Field M. New insights into Brunner syndrome and potential for targeted therapy. Clin Genet. Jan 2016; 89(1):120-127.
  3. BRUNNER SYNDROME. OMIM. June 2014; http://www.omim.org/entry/300615.
  4. Godar SC, Bortolato M, Castelli MP, Casti A, Casu A, Chen K, Ennas MG, Tambaro S, Shih JC. The aggression and behavioral abnormalities associated with monoamine oxidase A deficiency are rescued by acute inhibition of serotonin reuptake. J Psychiatr Res. September 2014; 56:1-9.
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Basic Information

  • Genetics Home Reference (GHR) contains information on Monoamine oxidase A deficiency. This website is maintained by the National Library of Medicine.

In Depth Information

  • Online Mendelian Inheritance in Man (OMIM) is a 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 Monoamine oxidase A deficiency. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Brunner syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.