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Diseases

Genetic and Rare Diseases Information Center (GARD)

Leukoencephalopathy with vanishing white matter


Other Names for this Disease
  • Childhood ataxia with diffuse central nervous system hypomyelination
  • CACH syndrome
  • CACH/VWM syndrome
  • Myelinosis centralis diffusa
  • Cree leukoencehalopathy
Related Diseases
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Treatment

Newline Maker

How might leukoencephalopathy with vanishing white matter be treated?

Treatment for leukoencephalopathy with vanishing white matter is supportive, aiming to alleviate symptoms. Management may include physical therapy and rehabilitation for motor dysfunction (mainly spasticity and ataxia); and anti-seizure medications for seizures.[1]

Infections and fevers should be prevented when possible through the use of vaccinations; low-dose maintenance antibiotics during winter months; antibiotics for minor infections; and antipyretics (fever-reducing medications) for fever. For children, wearing a helmet outside can help minimize the effects of head trauma. Contact sports, head trauma, and stressful situations (including high body temperature) should be avoided.[1]

More detailed information about the management of leukoencephalopathy with vanishing white matter is available on the GeneReviews Web site.
Last updated: 9/14/2015

References
  1. Raphael Schiffmann, Anne Fogli, Marjo S van der Knaap, and Odile Boespflug-Tanguy. Childhood Ataxia with Central Nervous System Hypomyelination/Vanishing White Matter. GeneReviews. August 9, 2012; http://www.ncbi.nlm.nih.gov/books/NBK1258/.


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.
Other Names for this Disease
  • Childhood ataxia with diffuse central nervous system hypomyelination
  • CACH syndrome
  • CACH/VWM syndrome
  • Myelinosis centralis diffusa
  • Cree leukoencehalopathy
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.