Juvenile Huntington’s Disease Linked to School and Social Problems

When children have problems at school, it is often linked to something bigger going on in another aspect of their life, whether that is at home or with friends. A study that was recently published in Brain Sciences evaluates how a rare disease, specifically juvenile Huntington’s disease, impacts children at school and in their social lives.

About Huntington’s Disease

Huntington’s disease is a progressive, neurological condition that causes the deterioration of the brain. It causes the loss of mental and physical functions. This disease is very rare, affecting three to seven of every 100,000 people. It tends to affect those of European ancestry, with diagnosis typically occurring from age 30 to 40. The HTT gene is mutated in those with this condition, and it creates the instructions for the protein huntingtin. The exact purpose of this protein is unknown, but doctors believe that it is involved in the health of neurons. It accumulates in long chains due to the mutation and it will then bind to neurons which lose function and die. This gene is inherited in an autosomal dominant pattern, meaning only one parent has to pass down the mutated gene for a child to be affected.

Huntington’s symptoms fall into one of three categories: emotional issues, cognitive decline, or uncontrolled motor symptoms. The first form of symptoms may manifest as personality changes, mood swings, anxiety, depression, and impulsive behavior. Cognitive decline sees effects such as poor decision-making, forgetfulness, and issues with learning and retaining new information. The last category includes symptoms like twitching, shrugging, and issues with walking, coordination, and swallowing. Chorea, or uncontrolled movements, is one of the most common effects. Genetic testing is the major method used to diagnose this disease, as people are typically aware that Huntington’s runs in their family. Once a diagnosis is obtained and symptoms begin, treatment is symptomatic. Anti-psychotic drugs or dopamine-depleting drugs are used to suppress chorea. Doctors may also prescribe anti-depressants, neuroleptics, and antiepileptic drugs.

While the symptoms of this disease do not typically appear until the third or fourth decade of life, there is a juvenile form in which individuals under the age of 21 are affected. This is the form that is being evaluated in this study.

About the Study

Researchers at Germany’s Ruhr-University Bochum decided to conduct a case series study in order to discover how much juvenile Huntington’s disease impacts school children; they also took into account how living with a parent with an earlier onset of the disease affects children.

They used the data from patients who had been treated at their Huntington’s center over the course of the past 25 years, finding that 32 patients were diagnosed before they were 21 (juvenile group), and 18 were diagnosed before age 18 (pediatric group). These numbers are very low, considering that 2,593 patients had been treated at the center.

Within the pediatric group, the age of onset was 10.3 years old, which can be compared to the juvenile group at 19.3 years old. Between the two groups, about 70% of patients inherited the disease from their father.

Researchers compared the symptoms presented in both groups and found that:

  • Pediatric patients experienced more muscle rigidity, tremors, and dystonia
  • Chorea was more common in the juvenile group
  • 37.5% of pediatric patients experienced seizures, compared to 22.2% of juvenile patients
  • Aggression, irritability, and other behavioral issues were present in 1/3 of the juvenile group and 62.5% of the pediatric group

When researchers investigated school and social problems, they found that children demonstrated a high degree of issues. These issues were often associated with cognitive decline and other symptoms of Huntington’s. Researchers also discovered that a high percentage of patients had difficult home lives, which also contribute to social and school problems.

In terms of treatment, the patients included in this study were given dopaminergic medications, cannabinoids, benzodiazepines, deep brain stimulation, botulinum toxin injections, clozapine, and zuclopenthixol. The majority of patients required an individualized approach to their treatment.

Something interesting that the researchers note is that the children were relieved by their diagnosis of Huntington’s disease. They felt that it finally explained all of the issues they were having in their social and school lives.

Read more about this study here.

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