Neuronal ceroid lipofuscinoses (NCLS)

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

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

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

The younger the person is when the disease appears, the greater the risk for disability and early death. Those who develop the disease early can have vision problems that progress to blindness, and problems with mental function that get worse. If the disease emerges in the first year of life, death by age 10 is likely.

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If the disease occurs in adulthood, symptoms will be milder with no vision loss and a normal life expectancy.

• Vision impairment or blindness (with the early-onset forms of the disease)
• Mental impairment, ranging from severe retardation at birth to dementia later in life
• Rigid muscles (due to severe problems with the nerves that control muscle tone)

The person may become totally dependent on others for help with daily activities.

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

Review Date: 10/14/2009
Reviewed By: Luc Jasmin, MD, PhD, Departments of Anatomy and Neurological Surgery, University of California, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org).