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Batten Disease and Childhood Dementia

Batten disease is a fatal, hereditary disorder of the nervous system that begins in childhood. Symptoms of Batten disease are linked to a buildup of substances called lipopigments in the body's tissues. Early symptoms of Batten disease include:
  • Personality and behavior changes
  • Slow learning
  • Clumsiness
  • Stumbling.
Over time, children who are affected with Batten disease suffer:
  • Mental impairment
  • Seizures
  • Progressive loss of sight and motor skills.
Eventually, children with Batten disease will develop childhood dementia and become blind and bedridden. The disease is often fatal by the late teens or 20s.

Childhood Dementia and Lafora Body Disease

Lafora body disease is a rare genetic disease that causes seizures, rapidly progressive dementia, and movement problems. These problems usually begin in late childhood or the early teens. Children with Lafora body disease have microscopic structures called Lafora bodies in the brain, skin, liver, and muscles. Most affected children die within 2 to 10 years after the start of symptoms.

Other Conditions Associated With Childhood Dementia

A number of other childhood disorders can include symptoms of childhood dementia. Examples of such conditions include:
  • Mitochondrial myopathies
  • Rasmussen's encephalitis
  • Mucopolysaccharidosis III (Sanfilippo syndrome)
  • Neurodegeneration with brain iron accumulation
  • Leukodystrophies such as Alexander disease, Schilder's disease, and metachromatic leukodystrophy.
Written by/reviewed by:
Last reviewed by: Arthur Schoenstadt, MD
Last updated/reviewed:
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