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- Medline Plus: Basal Ganglia Dysfunction
- GeneReviews: Huntington Disease
- GeneReviews: Huntington Disease
- National Institute of Neurological Disorders and Stroke: Huntington's Disease
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Huntington's disease is an inherited degenerative disorder of the brain characterized by loss of muscle control and higher brain functions 2. Symptoms typically begin in adulthood with progressive deterioration occurring over a 13 to 15 year period. People with late-stage Huntington's disease manifest symptoms associated with a significant loss of brain tissue, especially in the basal ganglia 2. These brain structures aid in the control of movement, memory and higher thought processes.
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Daily Living Dependence
Huntington's disease adversely affects the control of involuntary and voluntary movement 2. In the final stage of the illness, people with Huntington's disease experience marked difficulty initiating and controlling any form of voluntary movement 2. The loss of volitional control of movement proves incapacitating. Patients require round-the-clock assistance for the basic functions of daily living such as dressing, eating and bathing. Loss of bladder and bowel control leads to urinary and fecal incontinence.
Destruction of the basal ganglia with loss of movement control, incoordination and postural disturbances leaves patients with advanced Huntington's disease wheelchair-bound 2. Patients often lack the strength and coordination to maneuver a wheelchair, creating a situation of complete mobility dependence. Patients in the final stages Huntington's disease are often bed-bound, as their rigidity, lack of body control and postural instability interfere with the ability to safely assume and maintain a sitting posture 2.
Inability to Speak
People with Huntington's disease slowly lose the ability to speak 2. In the final stage of the illness, the cumulative effects of the inability to initiate movement, lack of coordination and dementia combine to destroy the capacity for spoken communication.
Huntington's disease adversely affects the ability to chew and swallow 2. Loss of voluntary muscle control also interferes with the ability to maneuver the hands for self-feeding. Progression of these symptoms typically leads to an inability to ingest sufficient food and liquids. Chronic weight loss develops as a characteristic symptom of late-stage Huntington's disease 2. Patients often require a feeding tube to maintain their nutritional intake.
Dementia is an impairment in the capacity for higher intellectual functions such as learning, reasoning, planning, social perception and adaptation. Huntington's disease causes progressive dementia that becomes severe in the late stages of the illness 2. Family members may no longer be recognized and intellectual function significantly regresses, severely limiting the ability to understand, perceive and interact. Huntington's disease may also cause psychiatric disturbances such as personality changes, obsessive-compulsiveness and anxiety 2.
Patients in the final stages Huntington's disease are often bed-bound, as their rigidity, lack of body control and postural instability interfere with the ability to safely assume and maintain a sitting posture 2. In the final stage of the illness, people with Huntington's disease experience marked difficulty initiating and controlling any form of voluntary movement 2. Destruction of the basal ganglia with loss of movement control, incoordination and postural disturbances leaves patients with advanced Huntington's disease wheelchair-bound 2.
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