Home > Medical Reference > Encyclopedia (English)

Toggle: English / Spanish

 

Video details

Do Not Remove This Text

Related Content


 

Neuronal ceroid lipofuscinoses (NCLS) - Treatment

Alternative Names

Lipofuscinoses; Batten disease; Jansky-Bielschowsky; Kufs' disease; Spielmeyer-Vogt

Treatment:

Treatment depends on the type and extent of symptoms. You may need lifelong assistance and care.

Support Groups:

For information and support, see www.bdsra.org.

Expectations (prognosis):

If the disease occurs in adulthood, symptoms will be milder with no vision loss and a normal life expectancy. Those who develop the disease early can have vision problems that progress to blindness, and mental deterioration. If the disease emerges in the first year of life, death is likely by age 10.

Complications:

People with the early-onset forms of the disease typically have visual impairment or blindness. Mental impairment can range from severe retardation at birth to late-onset dementia. There can be severe problems with the nerves that control muscle tone, making the muscles rigid. The person may become totally dependent on others for help with daily activities.

Calling your health care provider:

Call your health care provider if your child shows symptoms of blindness or retardation.

  • Reviewed last on: 10/25/2007
  • Chad Haldeman-Englert, M.D., Division of Human Genetics, Children's Hospital of Philadelphia, Philadelphia, PA. Review provided by VeriMed Healthcare Network.