Juvenile Huntington Disease (JHD) is a form of Huntington’s disease, identified initially by particular signals and indicators prior to a person reaching twenty years of age. Exact universality of the juvenile form is not known, but it is approximated to be about 1 in every 1600,000 youths.

The symptoms are somewhat dissimilar from the adult form. Children who have earlier onset of the illness are more probable to be very stiff and less likely to have involuntary motions as seen in adults with Huntington’s disease.

Children with JHD will have increased learning problems, may have serious behavioral troubles, and proneness to experience convulsions and epileptic seizures, something uncommon to adults suffering from the disease.

Juvenile Huntington’s disease is extremely infrequent, affecting less than 1/10 of all people with Huntington’s disease. Additionally, as the symptoms can be very dissimilar from those in adults, coping with JHD can be an alienating experience for the adolescents suffering from HD, their families and the professionals involved.

JHD is an intensifying disorder that causes the malfunction of brain cells in certain sectors of the brain. This results in undisciplined movements, loss of mental abilities and psychological disturbances. JHD is inherited in an autosomal dominant pattern and is generated by a large number of CAG trinucleotide repeats in the HTT gene. A great amount of repeats is usually related to an earlier onset of signs and symptoms. Most often, children with juvenile HD inherit the developed CAG trincleotide repeat from the father, however on occasion they can inherit it from the matriarch.

As previously stated, the majority of children suffering from HD with very expanded mutations inherit them from their fathers, this could be attributed to the varying timetables of sperm and egg production. A female at puberty has around 400,000 eggs, each stopping on the brink of completing meosis, when the slippage that expands the gene could happen, compared to a male who releases a quarter of a billion sperm each ejaculation therefore creating many chances over a reproductive lifetime for the gene to be miscopied and expand.

An exam to calculate CAG repeats is vital to confirm a clinical diagnosis of HD. When genetic testing first became accessible in the 1990’s there was concern that people discovering they had the mutation prior to symptoms would become anxious & upset. That hasn’t occurred but neither has testing did not become very popular either Most individuals, who know they have one affected parent generally choose not to be tested, however when a young person has symptoms it is considered necessary for the testing to occur.

As for research and conquering HD, CHDI (Cure Huntington’s Disease Initiative), HDSA (Huntington’s Disease Society of America) and others finance a vast amount of research into developing treatments for Huntington disease; from cell biology to population studies that track the diseases progression/regression with the overall intention of eradicating the disease altogether… or at the least finding methods to treat it.

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