Fast Five Quiz: Huntington Disease

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

Disclosures

May 27, 2020

Figure 1. This image shows slices from two brains. On the left is a normal caudate; on the right, a brain from a patient with Huntington's Disease shows severe atrophy of the caudate.

The frequency of Huntington disease in different countries varies widely. This is probably a result of a founder effect, which is defined by reduced genetic diversity in a population descendant from a small number of colonizing ancestors.

The most common age of onset is during adulthood, with a mean age of onset ranging from 35 to 44 years. Huntington disease is an autosomal dominant inherited disorder, which therefore means that each child of an affected parent has a 50% chance of developing the disease.

Huntington disease is associated with cell loss within a specific subset of neurons in the basal ganglia and cortex. The most conspicuous neuropathology occurs within the neostriatum, including gross atrophy of the caudate nucleus and putamen along with selective neuronal loss and astrogliosis.

Learn more about the epidemiology of Huntington disease.

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