Huntington's Disease Introduction & Overview

Conditions & Diseases: Brain & Nervous System

Huntington's Disease

Introduction & Overview

Huntington’s disease (HD) is a fatal, hereditary, progressive, neurodegenerative disorder characterized by gradual development of emotional, behavioral, psychiatric abnormalities with loss of intellectual or cognitive functioning and movement abnormalities such as uncontrolled jerking movement of the limbs, trunk and the face (chorea) (1) & (2). In simple terms, it is a genetic disorder of the central nervous system which leads to involuntary movements, dementia, and eventually, death.

In the past, this disorder was called chorea (derived from the word Choreography – Greek meaning dance), later it was called various other names such as hereditary chorea, dancing disease, chronic progressive chorea, degenerative chorea and now it is called as Huntington’s disease (named after Dr.George S. Huntington who discovered that it was a hereditary disorder) (3).

Juvenile Huntington’s disease is also called Westphal variant or akinetic-rigid Huntington’s disease which develops before a child or teen reaches their 20th birthday.

Juvenile Huntington's disease progresses rapidly with muscle rigidity resulting in limited movement capabilitiy of the patient. It is important to note that every child of a parent suffering from HD is at 50-50 risk of inheriting the disease (1).

Approximately one in every 10,000 people develops HD and there are approximately 30,000 total people suffering from this disorder in the US. Approximately 150,000 people in the US are at risk of developing HD for genetic reasons.

Huntington’s disease is more prevalent in whites as compared to African-Americans in the US and usually strikes people 30 to 50 years of age.

Article by Kona Vishnu, MS
Medical Writer,
OmniMedicalSearch.com