Huntington’s Disease Effects

Huntington’s disease is a genetically transmitted disease that primarily affects the nervous system. It is characterized by loss of neurons in certain parts of the brain, especially the caudate nucleus and putamen regions. Symptoms typically begin between the ages of 30 and 55 (see Goldman) and can include a wide variety of symptoms. The diagnosis is made by genetic testing, and sadly, the prognosis is poor. While medications are available for treatment, from its onset, the disease progresses to institutionalization and death over the course of 15 years.

Motor Symptoms

The body’s motor functions are markedly affected in Huntington’s disease. The most common type of dysfunction is known as chorea, and is characterized by quick movements of the feet or hands that are commonly compared to dance-like motions. Parkinson-type symptoms such as slowed movements and gait tremor also can develop (see Goldman) in addition to a disorder known as dystonia. Dystonia is characterized by sustained muscle contractions that can cause repetitive movements and/or abnormal body posturing. These are all related to the loss of neuronal cells in the caudate nucleus part of the brain.

Cognitive Deterioration

The loss of brain cells also affects the person’s cognitive skills. Memory is not as badly affected as the ability to plan and organize internal thoughts. Symptoms include altered attention, diminished concentration and deficits in judgment and problem solving. Medications are available that can alleviate this problem but inevitably it progresses to dementia.

Psychiatric Symptoms

Psychiatric manifestations of disease are almost universal in patients afflicted with Huntington’s disease. They most commonly include personality changes, increased impulsiveness, psychotic symptoms, depression, and increased aggressiveness. Often these symptoms can appear before the motor symptoms, which can make diagnosis quite challenging.

Weight Loss

Weight loss is also found in almost all patients with Huntington’s disease but is not necessarily associated with the damage to the nervous system. It is poorly understood but according to an August 2009 article in the Lancet Neurology journal, patients with a higher body mass index at disease onset tend to have a slower rate of disease progression. One possible cause for this has been involvement of immune cells that release inflammatory chemicals in the bloodstream. Chronically elevated levels of inflammatory chemicals seems to cause the atrophy and weight loss found in Huntington’s disease. This has been postulated to be a future area of medical intervention.

Muscle Atrophy

Diminished skeletal muscle is also a hallmark of Huntington’s disease, despite their relative hyperactivity due to the movement disorders. The genetic defect that causes neuronal loss has also been theorized to directly affect muscle cells and muscle mass.

Cardiac Failure

A leading cause of death in Huntington’s disease patients is heart failure, occurring in thirty percent of patients according to the Lancet Neurology journal. The same genetic defect that affects skeletal muscle also seems to affect cardiac muscle, thus resulting in failure of heart function.

About this Author

Saad Mohammad, M.D., is currently in the process of applying to residency anesthesiology. His first published research occurred in 2009 at the American Thoracic Society National Conference where a poster presentation was accepted and at the Bridgeport Symposium 2009. He began professionally writing in 2009 for Demand Studios as a freelance writer on issues in health and medicine.