Batten Disease Symptoms
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- What It Is
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Symptoms of Batten Disease
Symptoms of Batten disease include the following:
- Vision loss (an early sign) and blindness
- Muscle incoordination
- Mental retardation or decreasing mental function
- Emotional disturbances or difficulties
- Seizures
- Muscle spasms
- Deterioration of muscle tone
- Movement problems
Symptoms of Batten disease are similar in each type of the disease. However, the time of appearance, severity, and rate of progression of symptoms can vary depending on the type of the disease. For example:
Infantile NCL (Santavuori-Haltia disease)-symptoms begin to appear between the ages of six months and two years, and progress rapidly. Children with this type generally live until mid-childhood (about age five), though some survive in a vegetative state a few years longer.
Late infantile NCL (Jansky-Bielschowsky disease)-symptoms begin to appear between ages 2-4, and progress rapidly. Children with this type usually live until ages 8-12.
Juvenile NCL (Spielmeyer-Vogt-Sjogren-Batten disease)-symptoms begin to appear between ages 5-8, and progress less rapidly. Those afflicted usually live until their late teens or early 20s; and in some cases, into their 30s.
Adult NCL (Kufs disease or Party's disease)-symptoms usually begin to appear before age 40. Symptoms progress slowly and are usually milder. However, this form of the disease usually does shorten a person's life span.
