Conference Coverage

Huntington’s Disease Symptoms Vary by Age of Onset

Earlier age of onset may be associated with higher severity of behavioral symptoms.


 

MIAMI—The greater the age of Huntington’s disease onset, the lower the likelihood that the patient’s major symptom type at disease presentation will be behavioral or cognitive, according to research presented at the Second Pan American Parkinson’s Disease and Movement Disorders Congress. Increasing age at onset also is associated with a higher likelihood that motor symptoms will be the major symptom type at disease presentation.

Examining Enroll-HD Data

A patient may have subtle changes in movement, thinking, or behavior as early as 20 years before the onset of Huntington’s disease. Allison Daley, a clinical research specialist at Ohio State University in Columbus, and colleagues noted anecdotally that the severity of behavioral symptoms was decreased in older patients with newly diagnosed Huntington’s disease, compared with younger patients. The group set out to investigate the relationship between behavioral symptoms and age at onset of clinical symptoms in Huntington’s disease.

Allison Daley

Ms. Daley and colleagues examined data for 8,714 participants registered in the Enroll-HD database as of 2016. They established three categories of patients based on age of onset. Early-onset Huntington’s disease was defined as onset at an age younger than 30. Earlier adult-onset Huntington’s disease was defined as onset between ages 30 and 59. Later adult-onset Huntington’s disease was defined as onset at an age above 59. Ms. Daley’s group examined the frequency and severity of behavioral symptoms at disease presentation in all three groups. They used the Clinical Characteristics form and the Problem Behaviors Assessment form to evaluate symptom presence and severity.

Motor Symptoms More Common in Late-Onset Disease

Of the entire sample, 4,469 participants had manifest Huntington’s disease. Motor symptoms were present in 42% of participants with early-onset Huntington’s disease, 50% of participants with earlier adult-onset Huntington’s disease, and 67% of participants with later adult-onset Huntington’s disease. Cognitive symptoms were recorded in 9% of the early-onset group, 10% of the earlier adult-onset group, and 4% of the later adult-onset group. Behavioral symptoms were observed in 26% of the early-onset group, 19% of the earlier adult-onset group, and 11% of the later adult-onset group.

A one-year increase in age at onset was associated with a 5.5% decrease in the odds of severe behavioral symptom of any type. In addition, a one-year increase in age at onset was associated with a 9.4% decrease in the odds of severe disorientation, an 8.8% decrease in the odds of severe delusions, a 6.8% decrease in the odds of severe obsessive–compulsive behavior, and a 5.8% decrease in the odds of severe apathy.

At baseline, 56% of participants had apathy, 55% had anxiety, 54% had irritability, 51% had depression, 44% had perseverative thinking, and 29% had anger or aggression.

“Specific behavioral symptoms, particularly disorientation, delusions, and obsessive–compulsive behaviors, may tend to manifest more severely in individuals with earlier age at onset. Earlier age at onset may be associated with higher severity of behavioral symptoms overall,” said Ms. Daley. “Further research exploring biologic mechanisms and environmental factors that interact with CAG repeat size to affect symptom presentation in Huntington’s disease at different ages at onset may help identify additional therapeutic strategies and individualized treatment approaches for Huntington’s disease.”

—Erik Greb

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