Huntington's Disease Disorder

Huntington's disease (HD) is an inherited disorder involving the loss of neurons (Gusella, 1991, p. 125). This premature neuronal destruction results in a variety of neurologic and psychiatric symptoms.

For several reasons, HD has been the object of much study. One is the fact that it appears to result from a fully dominant, autosomal mutation. In addition, HD's age of onset is unusually variable. Finally, it seems that different forms of HD depend on which parent provided the mutant gene (Laird, 1990, p. 242).

Although HD had been known previously, widespread knowledge of the disease came as a result of work performed by Dr. George Huntington (LaCour, 1990, p. 16). Using records kept by his father and grandfather, Dr. Huntington elucidated the genetics behind HD.

The onset of HD symptoms is insidious (Gusella, 1991, p. 126). The diagnosis depends on a family history of the disease, the presence of a progressive voluntary movement disorder, and the patient's psychiatric disturbance (LaCour, 1990, p. 16). Different forms of HD vary in their severity: severity tends to decrease with later ages of onset.

Epidemiological studies of HD show that it occurs in all populations. However, it tends to be most prevalent in Caucasians of Western European origin; its incidence in this population is approximately 1 in 10,000 (Leung et al., 1992, p. 681).

Treatment consists of addressing the symptoms. DopamineD2antagonists may be used

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combined loss of memory and an impaired intellectual capacity (Gusella, 1991, p. 126). Eventually, motor dysfunction leads to speech problems. As communication with the victim of Huntington's disease becomes more difficult, it only acts to accentuate their observed mental problems (Gusella, 1991, p. 126). Eventually, practically all communication these patients becomes impossible. Besides having an insidious onset, Huntington's disease also has a variable onset (Gusella, 1991, p. 126). Averages ages at which the disease starts range from 35 to 42 years (LaCour, 1990, p. 21). Age of onset may also vary widely between members of the same family (Gusella, 1991, p. 126). The mean age in 243 patients studied in New England was as old as 41 years (LaCour, 1990, p. 21). The typical duration of Huntington's disease is about 17 years (Gusella, 1991, p. 126). However, some patients have survived as long as 30 years. These fortunate few eventually succumb to some secondary complications resulting from the original HD. Common secondary complications include heart disease and pneumonia (Gusella, 1991, p. 126). The extremely rare form of HD, very early onset HD, may appear in children younger than 10 years of age (Clarke & Bundey,

Category: Medical - H