Neuronal ceroid lipofuscinoses (NCLS)

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

Overview

Symptoms

Treatment

Prevention

Neuronal ceroid lipofuscinoses (NCLS)

Alternative Names:

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

Treatment:

Treatment depends on the specific symptoms and their extent. There may be a need for life-long assistance with care.

Support Groups:

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

Expectations (prognosis):

Progressive visual disturbance to blindness
Mental deterioration
Probable death by the age of 10 if the disease is apparent in the first year of life
Milder symptoms with no loss of vision and a normal life expectancy if disease is adult onset

Complications:

There is typically visual impairment or blindness in early-onset forms of the disease. Mental impairment can range from severe retardation at birth to only late-onset dementia. There can be severe problems with the nerves that control muscle tone, resulting in rigidity. 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.

Review Date: 11/21/2005
Reviewed By: Christos Ballas, M.D., Attending Psychiatrist, Hospital of the University of Pennsylvania, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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