Huntington's disease impairs the functioning of the brain, which can result in apathy, trouble organizing, impulsivity, irritability and anger, unawareness, disinhibition, preservation, and other psychiatric symptoms. These emotional and behavioral symptoms can further complicate the caregiver's role.
The behavioral changes vary from patient to patient, but often include apathy, marked by a loss of motivation to start or finish activities. Patients may be irritable or depressed. They may lack inhibition, and do or say things that one would normally find embarrassing.
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.
These patients often display a range of psychiatric symptoms, for example hallucinations or low mood, which coexist with behavioural change and symptoms such as apathy, irritability and aggression.
Depression and suicide are common among those with Huntington disease. Antidepressants and antianxiety medications may be prescribed to treat these symptoms.
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.
Impaired social functioning is a key feature of Huntington's disease (HD). In addition to motor limitations that prevent social participation (e.g., fall risk, driving cessation), personality changes caused by cognitive and psychiatric decline alter social functioning capacity in people with HD.
Neuropsychiatric symptoms are often present well before the motor symptoms manifest, sometimes decades prior, and include depression, irritability/aggression, executive dysfunction (eg, apathy, obsessive-compulsive behaviors), psychosis, cognitive decline, and dementia.
Familial prion disease may produce a diverse range of phenotypes, even within the same pedigree. It may resemble HD with prominent personality change, psychiatric symptoms and cognitive decline, chorea, rigidity, and dysarthria.
Early in the disease, cognitive decline may manifest as memory and learning difficulties, judgment impairment, and trouble with driving, answering questions or making decisions. As the disease progresses, concentration and focus on intellectual tasks become increasingly difficult.
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.
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder that usually starts in mid-adult life. The clinical disease progresses to death over an average of 20 years.
Mood and behavioral changes
Agitation, irritability, and aggression are other possible personality changes. Some patients may experience hallucinations and delusions that can severely affect their day-to-day interactions. Living with Huntington's can induce feelings of anxiety, depression, apathy, and frustration.
During the early stages of HD, irritability, depression, anxiety and aggression are common behavior symptoms. In the later stages, individuals tend to be less irritable and aggressive, as apathy and a lack of concern become more pronounced.
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.
Unawareness of motor, cognitive, behavioral, and functional aspects of HD has been documented throughout the disease course. This can occur at motor and cognitive onset but is more pronounced as the disease progresses.
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.
For individuals who are at risk of carrying the HD gene, testing can be performed before symptoms occur. Information from brain scans such as computed tomography (CT), electroencephalography (EEG) and magnetic resonance imaging (MRI) may be reviewed as part of the diagnosis.
It's hallmark motor sign is chorea: the occurrence of rapid, irregular, and arrhythmic complex involuntary movements. Along with chorea, substantial impairment of voluntary movement also occurs1, and it may be of greater functional importance in the lives of patients5.
Stage 1: Early Stage
Involuntary twitches in the toes, face or fingers. Struggling to concentrate or solve problems. Low coordination. Issues with doing complex movements.
HD patients present a broad range of psychological disturbances such as cognitive rigidity, mood disturbances, lack of empathy and breakdowns of social relationships that might also manifest even before the onset of motor dysfunction (Marvel and Paradiso, 2004).
Symptoms typically emerge from age 30 to 50, but also can develop in children and older adults. Late-onset Huntington's, characterized by some as emerging after age 5o and others after age 60, is thought to be less severe than earlier onset Huntington's.
Although not directly related to HD, stress is nevertheless related to the progression of the disease because it adds to the neurodegeneration that is already taking place. Chronic stress can alter nerve cells, brain structure, and brain function.