Huntington's disease is a condition that damages nerve cells in the brain causing them to stop working properly. It's passed on (inherited) from a person's parents. The damage to the brain gets worse over time. It can affect movement, cognition (perception, awareness, thinking, judgement) and mental health.
Huntington's disease is a progressive brain disorder caused by a single defective gene on chromosome 4 — one of the 23 human chromosomes that carry a person's entire genetic code. This defect is "dominant," meaning that anyone who inherits it from a parent with Huntington's will eventually develop the disease.
Huntington's disease (HD) is an inherited disorder that causes nerve cells (neurons) in parts of the brain to gradually break down and die. The disease attacks areas of the brain that help to control voluntary (intentional) movement, as well as other areas.
The first symptoms of Huntington's disease often include: difficulty concentrating. memory lapses. depression – including low mood, a lack of interest in things, and feelings of hopelessness.
Overview. Huntington's disease is a rare, inherited disease that causes the progressive breakdown (degeneration) of nerve cells in the brain. Huntington's disease has a wide impact on a person's functional abilities and usually results in movement, thinking (cognitive) and psychiatric disorders.
Huntington's disease (also known as Huntington disease) is a neurological (nervous system) condition caused by the inheritance of an altered gene. The death of brain cells in certain areas of the brain results in a gradual loss of cognitive (thinking), physical and emotional function.
Depression and behavioral disorders
The most painful conditions in Huntington's, said Achterberg, are limb pain, headache, abdominal pain and fractures.
The disease is staged based on your motor function and ability to complete everyday tasks. Typically, HD progresses for 10 to 30 years. Most people with HD die from complications related to the disease. There is no cure for HD, and current medications can only relieve its symptoms, not slow or delay the progression.
The symptoms usually start at 30 to 50 years of age, but can begin much earlier or later. Symptoms of Huntington's disease can include: difficulty concentrating and memory lapses.
In 1872, George Huntington gave the first complete description of the disease based on his studies of several generations of one family who exhibited similar symptoms.
Huntington's disease is caused by a faulty gene. Cells in parts of the brain are very sensitive to the effects of the faulty gene. This makes them function poorly and eventually die. A parent with the Huntington's disease gene has one good copy of the gene and one faulty copy.
The overall presentation of HD was considered to be initially mild by most families in this study, but progression in motor and/or behavior symptoms eventually occurred in all 30 subjects evaluated more than once.
Although no disease-modifying therapies currently exist to slow or halt the progression of Huntington's disease, many new types of treatment are under investigation that may offer hope for the future.
Your provider may order brain-imaging tests for assessing the structure or function of the brain. The imaging technologies may include MRI or CT scans that show detailed images of the brain. These images may reveal changes in the brain in areas affected by Huntington's disease.
Huntington's is a progressive disease. Once the symptoms appear, they continue and worsen over time. In most cases, they become noticeable between the ages of 30 and 50 and typically begin gradually. Although their onset and progression can vary from person to person, they fall into three basic categories.
Lots of people at risk of Huntington's disease decide they'd rather not know until any symptoms appear. If you do want to know, ask your GP for a referral to a genetic counsellor. You'll have several appointments with the counsellor.
From the onset of symptoms, people with HD have a life expectancy of 10 to 25 years. HD is not evident at birth. In fact, symptoms will usually not appear until a person is between 35 and 55 years of age, and occasionally even later in life.
In addition, neuroimaging showed that regional brain atrophy was not as rapid in those receiving treatment, evidence that creatine might slow the progression of Huntington's disease before symptoms develop.
Weight loss can make symptoms worse and weaken the patient's immune system, making them more vulnerable to infections and other complications. Huntington's disease itself is not usually fatal, but it can lead to choking, pneumonia, or other infections that can lead to death.
Pneumonia and heart disease are the two leading causes of death for people with HD.
When alcohol becomes a coping mechanism for Huntington's disease, that's when it becomes a problem. “With any neurodegenerative or medical disease, drinking a lot can certainly affect your balance and your gait,” says Dr. Oguh.
Early signs and symptoms can include irritability, depression, small involuntary movements, poor coordination, and trouble learning new information or making decisions. Many people with Huntington disease develop involuntary jerking or twitching movements known as chorea.
Pneumonia: Difficulty swallowing and decreased mobility can increase the risk of pneumonia, including aspiration pneumonia. Infections: Infections can develop as a result of the overall decline in health and as a result of diminished self-care.