Degenerative changes in Huntington's chorea are seen in
Huntington's disease (HD), also known as Huntington's chorea, is an inherited disorder that results in the death of brain cells.The earliest symptoms are often subtle problems with mood or mental abilities.] A general lack of coordination and an unsteady gait often follow.As the disease advances, uncoordinated, jerky body movements become more apparent. People with HD often underestimate the degree of their problems Symptoms of HD most commonly become noticeable between the ages of 35 and 44 years, but they can begin at any age from infancy to old age.In the early stages, there are subtle changes in personality, cognition, and physical skills. are generally not severe enough to be recognized on their own at the earlier stages. Sleep disturbances are also associated symptoms. Especially affected are executive functions, which include planning, cognitive flexibility, abstract thinking, rule acquisition, initiation of appropriate actions, and inhibition of inappropriate actions.[As the disease progresses, memory deficits tend to appear. Repoed impairments range from sho-term memory deficits to long-term memory difficulties, including deficits in episodic (memory of one's life), procedural (memory of the body of how to perform an activity) and working memory.[Cognitive problems tend to worsen over time, ultimately leading to dementia.[This pattern of deficits has been called a subcoical dementia syndrome to distinguish it from the typical effects of coical dementias e.g. Alzheimer's disease.[ Repoed neuropsychiatric manifestations are anxiety, depression, a reduced display of emotions (blunted affect), egocentrism, aggression, and compulsive behavior, the latter of which can cause or worsen addictions, including alcoholism, gambling, and hypersexuality.[Difficulties in recognizing other people's negative expressions have also been observed.[ The prevalence of these symptoms is highly variable between studies, with estimated rates for lifetime prevalence of psychiatric disorders between 33% and 76%.[For many sufferers and their families, these symptoms are among the most distressing aspects of the disease, often affecting daily functioning and constituting reason for institutionalization.[Suicidal thoughts and suicide attempts are more common than in the general population.[Often individuals have reduced awareness of chorea, cognitive and emotional impairments.[ Mutant Huntingtin is expressed throughout the body and associated with abnormalities in peripheral tissues that are directly caused by such expression outside the brain. These abnormalities include muscle atrophy, cardiac failure, impaired glucose tolerance, weight loss, osteoporosis, and testicular atrophy. Ref Harrison20th edition pg 2677
Cerebellum
Caudate nucleus
Red nucleus
Pons
C.N.S