In 1872, a physician from Long Island, New York named George Huntington described a medical condition characterized by jerky, involuntary movements. Now known as Huntington disease, the condition typically appears in middle age. The initial symptoms are subtle, consisting of mild behavioral and neurological changes; but, as the disease progresses, speech is impaired, walking becomes difficult, and psychiatric problems develop that frequently lead to insanity. Most people who have Huntington disease live for 10 to 30 years after the disease begins; there is currently no cure or effective treatment.
Huntington disease appears with equal frequency in males and females, rarely skips generations and, when one parent has the disorder, approximately half of the children will be similarly affected. These are the hallmarks of an autosomal dominant trait — with one exception. The disorder occasionally arises before the age of 15 and, in these cases, progresses much more rapidly than it does when it arises in middle age. Among younger patients, the trait is almost always inherited from the father. According to Mendel's principles of heredity (Chapter 3), males and females transmit autosomal traits with equal frequency, and reciprocal crosses should yield identical results; yet, for juvenile cases of Huntington
Was this article helpful?