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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Leukoencephalopathy with vanishing white matter


Other Names for this Disease

  • CACH syndrome
  • CACH/VWM syndrome
  • Childhood ataxia with diffuse central nervous system hypomyelination
  • Cree leukoencehalopathy
  • Myelinosis centralis diffusa
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Treatment

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How might leukoencephalopathy with vanishing white matter be treated?

There is no cure for this condition. Treatment is usually supportive, seeking to alleviate symptoms associated with this condition. The following treatments may be necessary:[1]

  • Physical therapy and rehabilitation for motor dysfunction (mainly spasticity and ataxia)
  • Ankle-foot orthotics in individuals with hypotonia and weakness of the ankles
  • Anti-seizure drugs for treatment of seizures and abnormalities of behavior and mood

Because fever may exacerbate symptoms of this condition, it is important to prevent infections and fever as much as possible which can be achieved by administering vaccinations (including anti-flu vaccinations), low-dose maintenance antibiotics during the winter time, antibiotics for minor infections, and non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen and aspirin for fever.[1]

Affected individuals should be closely monitored for several days following head trauma or a major surgical procedure with anesthesia because neurologic deterioration (presumably stress related) may follow.[1]
Last updated: 4/1/2011

References
  1. Schiffmann R, Fogli A, Van der Knaap MS, Boespflug-Tanguy O.. Childhood Ataxia with Central Nervous System Hypomyelination/Vanishing White Matter. GeneReviews. February 2010; http://www.ncbi.nlm.nih.gov/books/NBK1258/#cach.Management. Accessed 4/1/2011.


Management Guidelines

  • GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the link to view the article on this topic.
Other Names for this Disease
  • CACH syndrome
  • CACH/VWM syndrome
  • Childhood ataxia with diffuse central nervous system hypomyelination
  • Cree leukoencehalopathy
  • Myelinosis centralis diffusa
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.