Batten disease

Batten disease is a rare, progressive, degenerated brain disease of childhood caused by a metabolic disorder in the processing of polyunsaturated fatty acids.

It is increasingly disabling, both physically and mentally and there is no cure. Most victims do not survive beyond the age of 10 years.

Basically, it is as if there were 800,000 people in one room. Only two carry the gene for late infantile Batten Disease. They meet, marry, and their first child together is affected.

Synonyms

• Stengel-Batten-Mayou-Spielmeyer-Vogt-Stock Disease
• Batten-Vogt Syndrome
• Batten-Mayou Syndrome
• Batten-Spielmeyer-Vogt's Disease
• Spielmeyer-Vogt-Batten Syndrome
• Stengel's Syndrome
• Neuronal Ceroid Lipofuscinosis, Juvenile Type
• Spielmeyer-Vogt Disease

Some facts about Batten disease

It is believed that the defective Batten's gene causes a deficiency of an essential enzyme (that is not yet identified). This enzyme is believed to be responsible for processing lipopigments out of brain cells.

Lipopigments are made up from Lipoproteins which are naturally present in brain cells, but only collect and remain there in Batten's victims. The lipopigments are thought to be responsible for the breakdown of the brain cells. But even this process is largely unknown and highly theoretical.

We are not scientists or researchers. This is how we best understand the process to occur.

Occurring mostly in families of Northern European Scandinavian ancestry, the disorder affects the brain and may cause both deterioration of intellect and neurological functions.