Juvenile Huntington disease

Disease Overview

Juvenile Huntington disease (HD) is a less common, early-onset form of Huntington disease that begins in childhood or adolescence. It is a progressive disorder that causes the breakdown of brain cells in certain areas of the brain. This results in uncontrolled movements, loss of intellectual abilities, and emotional disturbances. Juvenile HD is defined by the onset of symptoms before age 20 years and accounts for 5-10% of all HD cases. It is inherited in an autosomal dominant pattern and is caused by a type of mutation called a trinucleotide repeat, in the HTT gene. Most often, children with juvenile HD inherit the mutation repeat from their fathers, although on occasion they inherit it from their mothers.[39][40] Juvenile Huntington disease has a rapid disease progression once symptoms present. There currently is no cure. Treatment is supportive and focused on increasing quality of life.[10901] Most people with juvenile HD do not survive more than 10 to 15 years after symptoms begin.[40]

Synonyms

• JHD
• Huntington disease, juvenile onset
• Juvenile onset HD
• Juvenile Huntington chorea