Fast Five Quiz: Huntington Disease Signs and Symptoms

Shaheen E. Lakhan, MD, PhD, MS, MEd


May 27, 2020

Figure 1. This axial (cross section) CT image of the brain shows advanced volume loss and ventricular enlargement in a 50-year-old patient.

In a case study by Jason and colleagues, cognitive manifestations were examined in relation to age, clinical onset, progression, and genetic analysis. The results demonstrated that the presence of more trinucleotide repeats was associated with earlier age of onset. This study also showed that cognitive impairment correlated with number of years affected but not age at onset.

Most studies show a mean age at onset ranging from 35 to 44 years. However, the range is large and varies from 2 years to older than 80 years. Onset in patients younger than 10 years and in patients older than 70 years is rare.

Juvenile Huntington disease (Westphal variant) is defined as having an age of onset of younger than 20 years, and is characterized by mild or even absent chorea, as well as parkinsonian features, dystonia, long-tract signs, dementia, and epilepsy.

Learn more about the onset of Huntington disease dementia.


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