Huntington’s disease (HD) is a hereditary disorder which causes nerve cells to degenerate. The disease typically affects people starting in their 30s or 40s, but it’s possible for younger people to be affected by juvenile Huntington’s disease.
Since the disease affects the brain and nerve cells, patients notice a variety of symptoms. Changes in behavior, problems with coordination, involuntary movements, cognitive difficulties, and problems speaking and swallowing are a few common signs. These symptoms present themselves in varying degrees for each patient.
People can live with Huntington’s disease for 10-25 years. In its earliest stages, the illness presents minimal disruptions to patients’ lives. They may be able to live independently for several years. In middle-stage Huntington’s disease, symptoms become more disruptive, and people will need additional help carrying out everyday tasks. In the final stages, patients need others to care for them.
1. Amnesia
Retrograde amnesia is a common symptom of Huntington’s disease. This form of amnesia makes it difficult for patients to remember events that happened before the onset of illness. Patients may have strong memories from early childhood and young adulthood, but they may have difficulty with faces, names, and facts.
Deficits caused by Alzheimer’s Disease and Huntington’s have both been evaluated on the Dementia Rating Scale in scientific studies. Researchers found that Huntington’s patients commonly demonstrated perseveration and initiation. In other words, patients had difficulty starting new tasks. They were likely to repeat gestures, speech patterns, and responses because of their memory loss.
2. Delusion
Delusions are considered a rare symptom for Huntington’s patients, but they do occur for between 2% and 11% of people. Delusional people experience thoughts about situations that aren’t real. Many of these thoughts are completely harmless and may go unnoticed by caregivers.
In some situations, patients develop paranoid delusions. Medication may be necessary to control this symptom if it becomes disruptive or frightening for the patient.
The signs that someone is suffering from delusions may not always be obvious at first. Increased irritability, difficulty sleeping, and agitation are possible indicators. Sometimes this symptom manifests as bipolar disorder, in which the delusions induce a manic state which is followed by depression.
3. Memory Loss
Since Huntington’s affects nerve cells in the brain, it can also make it difficult for patients to encode new information or tackle multi-step processes. In addition to suffering from amnesia, patients appear to have a short attention span, and they may experience short-term memory loss.
Many patients seek help when they notice that they have trouble with recall. Loved ones may observe that a person is either struggling to produce general information or repeating something they’ve just said.
Memory loss can be associated with aging or illnesses including Alzheimer’s and dementia. A family history of Huntington’s disease or the presence of other symptoms can help doctors determine if memory loss is, indeed, a symptom of Huntington’s disease.
4. Confusion
Confusion is another of a multitude of cognitive symptoms. Forgetfulness and psychotic symptoms all contribute to a confused state. Since patients have difficulty taking in new information or solving problems with multiple steps, it’s hard for them to start new tasks and stay on track.
Difficulties with organization and prioritization make it tough for people to figure out which steps they need to take first in a series of activities. In mid and late-stage Huntington’s disease, things that seem simple, such as knowing to put on shoes after donning socks and pants can prove challenging.
Dementia-like symptoms cause people experience their world differently than when we’re healthy. A patient may only hear fragments of verbal instructions, which can increase their confusion.
5. Slowness
This symptom manifests in several ways. Cognitively speaking, Huntington’s disease patients need more time to process new information. They also have trouble thinking of the right word or remembering names. Slowness may be especially frustrating for the patient in the early stages when they are working to maintain their independence.
Huntington’s also causes patients to slow down physically. Since Huntington’s disease affects the basal ganglia, it impairs motor functions. Patients must move slowly to maintain their balance or compensate for erratic movements caused by their illness. Poor coordination can impair a person’s mobility as the disease progresses.
6. Abnormality Walking
Abnormality walking is a sign that most HD patients will experience. Difficulties with walking and coordination often occur in the early stages of the disease, and they are almost guaranteed in mid and late-stage Huntington’s disease. Patients may experience swaying, their gait may have an abnormal rhythm, and their knees may spontaneously flex. Patients may also notice that their stride length varies. Moving from side to side or having difficulty initiating a walking motion are other common gait anomalies.
All of this translates into a gait that appears stilted, uneven, or unstable. Involuntary motions also disrupt a patient’s gait.
7. Involuntary Movements
Involuntary movements are another common physical symptom of Huntington’s disease. As the basal ganglia are damaged by the disease, involuntary movement, along with abnormal gait and slowness, become progressively worse.
These dance-like motions are sometimes referred to as “chorea.” For some, the involuntary motions may be slight, such as tapping of fingers or sticking out the tongue. As degeneration continues, jerky motions can also affect the limbs, face, and trunk.
Early stage involuntary movements might appear to be minor tics to the observer. As the illness progresses, these abrupt and erratic movements can affect different parts of the body. This symptom eventually leads to loss of mobility and can make it difficult for patients to swallow.
8. Muscle Spasms
Muscle spasms, like abnormality walking and chorea, are common physical signs of HD. Huntington’s disease affects muscles on a cellular level. Muscular damage can cause atrophy, especially as the disease progresses. Even as atrophy weakens the muscles, cellular dysfunction can also result in painful spasms.
Patients may have to cope with minor spasms and tics, but they may also have prolonged periods of intense spasming. Spasms most often affect the limbs and face. In late stages of the illness, the severity of the spasms increases, and they become more common than involuntary movement.
When muscle spasms become bothersome, there are medications that can help to control this symptom.
9. Fidgeting
The causes for fidgeting are two-fold. On one hand, chorea, those involuntary motions so common among HD patients, can cause fidget-like symptoms. A patient’s fingers, hands, and feet may tap, which gives the appearance that they are fidgeting.
Like many of the physical symptoms of Huntington’s disease, this sign is often tied to the loss of motor control as the disease destroys nerve cells.
In addition to the physical causes of fidgeting, this symptom may manifest as a sign of anxiety. Patients with Huntington’s disease can experience psychotic behaviors, which may lead to restlessness. A doctor can help determine whether the fidgeting is a physical or a behavioral response to Huntington’s disease.
10. Hallucination
While some patients combat delusional thinking, they may also hallucinate. Hallucinations involve seeing, feeling, and hearing things that aren’t there. This symptom is rare compared to other signs on this list.
In some cases, hallucinations may be harmless to the patient. In other situations, the hallucinations are frightening or disturbing. A person might have a pleasant conversation with a long-lost loved one, but they may also experience unpleasant sensations, like bugs crawling over them. In cases where hallucinations cause fear and agitation, it may be necessary to seek medical attention to control this symptom.
11. Paranoia
A series of cognitive and behavioral changes manifest in HD patients. Between 6% and 25% of HD patients experience paranoia and other schizophrenia-like symptoms. Paranoid people are usually distrustful and suspicious of other people and the environment for unexplained reasons. Paranoia is often related to other psychotic signs of Huntington’s disease.
Paranoia is more common in early-onset Huntington’s disease. One study describes a patient who experienced the symptoms of schizophrenia several years before physical manifestations of Huntington’s appeared.
Doctors can administer antipsychotic drugs to curb paranoia. A medical team will be able to recommend medications that control a patient’s most troubling symptoms.
12. Anxiety
People learning to live with HD undergo a great deal of stress. A general uncertainty about the future, constant changes in abilities, and an increased dependence on others drive some patients to experience anxiety. They may also feel embarrassed about the physical signs of their illness, such as involuntary movements.
As HD progresses and patients lose cognitive function, the world becomes more frightening for them. They can’t always understand what is happening, and they may have trouble completing basic tasks. This aggravates anxious feelings.
One of the best ways to minimize anxiety for patients is the maintain a predictable schedule in a familiar setting. For severe anxiety, doctors may recommend medication.
A diagnosis of Huntington’s is difficult for patients and their loved ones. Although there is no cure, medications to control various manifestations of the illness enable patients to maintain their autonomy and have a good quality of life for longer than ever before. Identifying the disease early gives patients the opportunity to work with a medical team to get proper support and address problems as they arise.
