the child receives two copies of the defective gene. One from
Mom and one from Dad.Each child has a one in four chance (25%)
of having Batten Disease. If the child only receives one copy
of the defective gene the child will be a carrier and not
develop the disease.
There are three other main types of Batten Disease. These are:
Late-Infantile, Juvenile and Adult. There are variants off the
four types of Batten Disease.
Generally the onset of Infantile Batten Disease occurs between
the ages of 6 months and 2 years. Symptoms include but are not
limited to loss of acquired skills,muscle weakness,seizures,spasms,
lack of stimuli (sounds,pain etc.) and visual impairment.
Some examples are the sudden inability to use their leg(s) and/or
arm(s), inability to chew and swallow solid foods, excessive crying
fits. Basically overnight losing the ability to walk, eat, talk,
hold things in their hands etc. The symptoms may occur slowly over
many months or occur one after another very rapidly.
Eventually the child will reach what some call a plateau. By
doctors and other professionals the child will be labeled as
being in a vegatative state.
From the various sites and literature I have read a child with
Infantile Batten Disease has the average life expectancy of
5 to 6 years of age. There are and has been children that have
exceeded the documented average life span. How each child passes
varies.
At this time there is no known treatment to stop or reverse
the symptoms of Infantile Batten Disease. Seizures can be
reduced or controlled with the use of medication.
Research is being done to find effective treatments to slow
down and halt Batten Disease. There is a strong need for more
public awareness of this terrible disease.
If you would like more information about Batten Disease,
interested in helping or know someone who needs help
please contact The Batten Disease Research and Support
Association at http://www.bdsra.org.
I hope I have been successful in giving you a general
description of Batten Disease. If you have any questions
please feel free to email me and I will do my best to
Infantile Batten Disease
(INFANTILE NEURONAL CEROID LIPOFUSCINOSIS)
Batten Disease is an autosomal recessive disorder. Which means
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