Neurological Disease/ Huntingtons Disease

Huntington 's disease

Huntington 's disease is a chronic , hereditary disease of the nervous system that results in progressive involuntary choreiform or dancelike movement and dementia . It affects men and women of all races . Because it is transmitted as an autosomal dominant genetic dis , each child of a parent with Huntington 's disease (HD ) has a 50 risk of inheriting the illness

Huntington 's disease affects all races and ethnic group . Both sexes are affected too . It was estimated by the experts that in every 10 ,000 persons in the United

States 30 ,000 have Huntington 's disease

The basic pathology involves premature death of cells in the striatum (Caudate and putamen ) of the basal ganglia , the region deep within the brain in the control of movement . There is also loss of cells in the cortex , the region of the brain associated with thinking , memory perception and judgment and in the cerebellum , the area that coordinates voluntary muscle activity . Researchers now believe that a building block from a protein , called glutamine , abnormally collects in the cell nucleus , causing cell death (Barinaga , 1996 . Why the protein destroys only certain brain cells is unknown . The cells destruction results in the lack of the neurotransmitters , gamma-aminobutyric acid and ecetycholine , which inhibit nerve action . Onset usually occurs between the ages of 35 and 45 years about 10 of patients are children . The disease progresses slowly . Despite a ravenous appetite , patients usually become emaciated and exhausted . Patients succumb in 10 to 15 years to heart failure , pneumonia or infection or as a result of fall or choking

The most prominent clinical features of the disease are abnormal involuntary movements (chorea , intellectual decline and often emotional disturbance

As the disease progress , a constant writhing , twisting , uncontrollable movement may involve the entire body . These motions are devoid of purpose or rhythm , although patients may try to turn them into purposeful movement . All of the body musculature is involved . Facial movements produce tics and grimaces . Speech is affected , becoming slurred , hesitant , often explosive , and eventually unintelligible Chewing and swallowing are difficult , and there is a constant danger of choking and aspiration . Choreiform movements persist but diminish during sleep

As with speech , the gait becomes disorganized to the point that ambulation eventually is impossible . Although independent should be encouraged for as long as possible , a wheelchair usually becomes necessary at some point . Eventually , the patient is confined to bed when the chorea interferes with walking , sitting and all other activities Cognitive function is usually affected , with dementia usually occurring Initially , the patient generally is aware that the disease is responsible for the myriad dysfunctions that are occurring . The mental and emotional changes that are occurring with HD may be more devastating to the patient and family than the abnormal movements . Patients may be nervous , irritable or impatient . In the early stages of the illness patients are particularly subject to uncontrollable fits of anger profound , often suicidal depression apathy or euphoria . Judgment and memory are impaired and dementia eventually...