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. Dopamine D2 antagonists may be used to control the chorea; whereas a variety of measures may be employed against the psychiatric manifestations (Birkmayer & Rinne, 1993, p. 19). To date, the exact mechanisms involved in HD remain to be determined.
The pathologic condition, Huntington's Disease (HD), involves the progressive deterioration of the central nervous system. This disease is inherited. In some families, it can actually be traced back over several generations. Overall, in the United States, the social and...