What are the types of Batten disease?

By  ,  National Institute of Health
Jan 09, 2013

There are three other main types of NCL, including two forms that begin earlier in childhood and a very rare form that strikes adults. The symptoms of these three types are similar to those caused by Batten disease, but they become apparent at different ages and progress at different rates.


•    Infantile NCL (Santavuori-Haltia disease) begins between about 6 months and 2 years of age and progresses rapidly. Affected children fail to thrive and have abnormally small heads (microcephaly). Also typical are short, sharp muscle contractions called myoclonic jerks. Patients usually die before age 5, although some have survived in a vegetative state a few years longer.


•    Late infantile NCL (Jansky-Bielschowsky disease) begins between ages 2 and 4. The typical early signs are loss of muscle coordination (ataxia) and seizures that do not respond to drugs. This form progresses rapidly and ends in death between ages 8 and 12.


•    Adult NCL (Kufs disease or Parry's disease) generally begins before the age of 40, causes milder symptoms that progress slowly, and does not cause blindness. Although age of death is variable among affected individuals, this form does shorten life expectancy

 

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