Early Onset Parkinson’s Disease: Find out more

A common myth is that one can only have Parkinson’s disease if they are elderly. However, this neurodegenerative disease is not entirely elusive to youth. Many between the ages 21 and 50 develop early-onset Parkinson’s disease. While the traditional form of the condition is well-known, early onset Parkinson’s disease is characterized by unique factors that affect symptoms, diagnosis, and management.

What is Early Onset Parkinson’s Disease?

Parkinson’s disease is a neurodegenerative disorder of the central nervous system. The central nervous system is made up of the brain and spinal cord. They communicate with nerves throughout the body through chemical messengers known as neurotransmitters. In Parkinson’s disease, nerve cells within the brain die off—interfering with the neurotransmitter dopamine. A lack of dopamine has an extensive impact on movement, cognition, and sleep.

Both forms of the disease follow the same biological mechanisms. However, early-onset Parkinson’s is characterized by symptoms appearing between the ages of 21 and 50 instead of 60 and older. Early-onset comprises 10 to 20 percent of all cases of Parkinson’s disease. 

Symptoms of Early Onset Parkinson’s Disease

The symptoms of Parkinson’s disease are universal. They vary in severity from patient to patient but are not as affected by age of onset.

Nonmotor Symptoms

• Orthostatic intolerance—A drop of blood pressure upon standing which results in dizziness, syncope (fainting), and pre-syncope (blackouts).
• Fatigue—Feeling excessively tired and lacking energy for daily activities.
• Sleep disorders—REM sleep behavior disorder is the most common sleep disorder that causes a person to “act out” their dreams. Insomnia, excessive daytime sleepiness, and restless leg syndrome are frequently reported by Parkinson’s patients.
• Constipation—Having fewer bowel movements than normal. 
• Loss of smell—Also called hyposmia, an early sign of Parkinson’s is losing a sense of smell.
• Speech problems—Speech may be slurred, slow, and low in volume. Stuttering can occur.
• Poor posture—Adopting a hunched, stooped posture due to muscle rigidity and other factors.
• Urinary incontinence—Signals from the brain do not reach the bladder and patients lose bladder control, experience urgency, and frequency. 

Motor Symptoms

• Resting tremor—Shaking in the fingers, feet, hands, or face that appears when the muscles are relaxed.
• Dystonia—Painful, involuntary contractions of the muscles which provoke twisting and abnormal postures.
• Muscle rigidity—Muscles all over the body are stiff and difficult to move.
• Bradykinesia—Slowed movement as a result of the reduction of spontaneous movement. Patients notice problems with repetitive activities such as brushing their teeth, changing their clothes, and walking.
• Postural instability—Impaired balance caused by a loss of reflexes that keep a person upright. It is challenging for Parkinson’s patients to close their eyes while simultaneously standing.
• Freezing—Freezing of gait is when patients feel as if their feet are glued to the floor. They remain stationary, cautious to move forward.
• Micrographia—Small and cramped handwriting.
• Loss of facial expression—Because of decreased facial movements, facial expression is less expressive. This symptom is described as “wearing a mask.” 

Cognitive Symptoms

• Memory loss—Parkinson’s disease interferes with the process of temporarily storing information, so memory loss is occasionally reported. Long-term memory remains relatively intact, but the act of recalling information is impaired.
• Confusion—Thoughts are unclear.
• Anxiety—Concern over life (i.e. family, finances, health, etc.) that is out of proportion to the situation.  
• Apathy—Reduced motivation and feelings of indifference.
• Psychosis—Disconnection from reality that manifests with hallucinations and delusions. 

Depression and Early Parkinson’s Disease

Depression is a state of sadness that persists longer than 2 weeks. It is a mental disorder accompanied by feelings of hopelessness, emotional suppression, mood swings, and low self-esteem.

Depression secondary to Parkinson’s disease is not unusual. Those with early-onset Parkinson’s disease are especially prone to depression. The American Academy of Neurology (1989) concludes, “a stepwise regression analysis showed that in the early-onset group, depression scores were significantly correlated with scores of cognitive impairment and duration of the disease.”

The disease drastically alters their lifestyle in terms of social relationships, family, and work. They are expected to remain independent throughout early and mid-adulthood, yet their condition makes them dependent on others for care. That is tough to handle emotionally, which contributes to the incidence of depression.

Distinguishing Factors of Early Onset Parkinson’s Disease

Parkinson’s disease presents similarly regardless of onset. However, early onset Parkinson’s disease differs in the following ways:

• Age of diagnosis—The age of diagnosis is 21 to 50 years old.
• Dystonia—Abnormal movements, like the involuntary twisting and postures, are more frequent in early onset Parkinson’s.
• Increased side effects from dopaminergic medications—Levodopa is a medication prescribed in the treatment of Parkinson’s. It is synthesized in the brain into dopamine. Over time, dopaminergic medications lead to dyskinesias, dystonia, and other abnormal muscle movements. Younger patients have increased susceptibility to these side effects.
• Less frequent cognitive symptoms—Memory loss, confusion, and eventual dementia are not as pronounced in early onset patients.
• Imbalance and incoordination—Those with early onset Parkinson’s are not as severely afflicted with balance and coordination issues.
• More likely to have a genetic cause—the cause of Parkinson’s disease is not entirely known. However, early onset is linked with genetic causes.

Progression of Early Onset Parkinson’s Disease

Perhaps the greatest distinguishing factor of early onset Parkinson’s disease is its progression. Studies show the average life expectancy for Parkinson’s disease patients diagnosed later is approximately 16 years with the age of death being 81. However, the progression is significantly slower for early onset Parkinson’s disease. Onset begins at an earlier age. Symptoms are subtle and gradually worsen over a period of time.

Parkinson’s disease progresses in a series of 5 stages based on the Hoehn and Yahr Scale:

1. Stage One—Symptoms are mild and unilateral (occurring on one side of the body). This includes intermittent tremors or changes in facial expression. With minimal impairment to daily function, patients in stage one do not seek medical attention immediately. If they do, physicians wait until further progression to make a formal diagnosis.
2. Stage Two—Stage one progresses to stage two in a matter of months to years. Involvement becomes bilateral (both sides of the body) but is still considered mild. Tremors, stiffness, and rigidity arise in the muscles, as does speech abnormalities and abnormal posture. Balance is not affected.
3. Stage Three—Although the patient remains independent in daily activities, stage three is moderate severity and patients are able to live alone. Balance and reflexes are hindered. Falls are a classic characteristic of this stage.
4. Stage Four—Parkinson’s is disabling in stage four. Mobility begins to go. Patients require assistance such as a walker or wheelchair to help them navigating walking, but they maintain some independence in other daily living activities and self-care tasks.
5. Stage Five—Symptoms are severely progressed. Cognition declines and can include hallucinations or delusions. Stage five of Parkinson’s demands 24/7 care, as patients cannot stand unassisted and freeze, stumble, and fall.

Not every patient progresses to the last stage. Early onset patients progress to the first stage at an average of 2.9 years in comparison to only 1.7 years in older onset. Symptoms do not always present in a specific order. Whereas motor symptoms are initially prominent in late onset, people with early onset Parkinson’s disease experience non-motor symptoms in the beginning stages of the disease.

Causes of Early Onset Parkinson’s Disease

Regardless of age, the causes of Parkinson’s disease are not entirely understood. Potential causes can be environmental factors such as exposure to toxins from industrial pollutants, herbicides, and chemical solvents, or it can be genetic. Early onset Parkinson’s has been associated with genetic mutations in the gene— GCH1 or guanosine triphosphate cyclohydrolase-1—that produces dopamine in the brain. Confirmed by research from Iowa State University, Caucasians with a GCH1 mutation developed symptoms of Parkinson’s five years earlier and had a 23 percent increased risk for the disease (Webb, 2017). Additionally, the National Parkinson Foundation states that 65% of people with onset between ages 20 and 30 likely possess a genetic mutation.

Diagnosing Early Onset Parkinson’s Disease

Early onset Parkinson’s disease is easily dismissed. Patients with the early onset form sometimes go years without a proper diagnosis because of their young age. Aside from having to undergo testing sooner, the diagnostic studies for early onset Parkinson’s disease are standard. There is no single test. Blood work and imaging are important to rule out alternative causes for symptoms.

Neurologists, physicians who specialize in the brain and nervous system, perform a thorough neurologic assessment for patients in which early-onset Parkinson’s disease is suspected. They review patient history, measure reflexes, and observe movement patterns. If the patient exhibits resting tremors or multiple non-motor symptoms, a clinical diagnosis of Parkinson’s is warranted.  

A dopamine transporter (DaTScan) might prove helpful. By injecting a radioactive tracer that attaches to the dopamine transporter, a DaTScan reveals irregularities in parts of the brain containing dopamine neurons. Most are diagnosed without a DaTScan.

Treatment for Early Onset Parkinson’s Disease

There is no cure for Parkinson’s disease. Treatment consists of a combination of therapies to manage symptoms and increase a patient’s quality of life. While treatment is similar despite the age of symptom onset, early onset Parkinson’s does influence the outcomes of the treatment options.

Medication

• Levodopa—Levodopa is a natural chemical in the brain that is converted to dopamine. It is an effective medication for Parkinson’s disease who have insufficient amounts of dopamine because it increases the availability of the critical neurotransmitter. In higher doses, levodopa causes nausea, orthostatic hypotension, and dyskinesias. Patients with early onset Parkinson’s experience more side effects.
• Dopamine agonists—Dopamine agonists also increase the amount of dopamine in the brain. Unlike levodopa, dopamine agonists mimic the role of dopamine in the brain rather than going through the conversion process.
• MAO B inhibitors—This class of drug prevents the breakdown of dopamine by inhibiting the enzyme monoamine oxidase B.
• Catechol O-methyltransferase (COMT) inhibitors— They extend the effect of levodopa that waxes and wanes as the disease progresses.
• Anticholinergics—Anticholinergics treat tremors and abnormal muscular movements, but physicians are cautious to prescribe them with the side effects of memory loss, hallucinations, confusion, dry mouth, and constipation.
• Amantadine—Prescribed in the early stages of Parkinson’s Amantadine controls the involuntary movements from levodopa.

Deep Brain Stimulation

Deep brain stimulation is a surgical procedure that sends electrical signals to the brain through surgically placed electrodes. The surgeon connects the electrodes to a generator implanted into the patient’s chest. This treatment is ideal for the early onset of Parkinson’s patients who respond poorly to dopaminergic medications. Deep brain stimulation combats medication fluctuations and targets motor symptoms like tremors, rigidity, slow movements, difficulty walking, and stiffness.

Physical Therapy

Exercise is a central component in the treatment of Parkinson’s disease. It focuses on improving flexibility, increasing muscle strength, and working on balance and coordination. Physical activity grows the neuronal connections in the brain to utilize dopamine to its fullest potential. Experts recommend at least 2.5 hours of exercise per week, which is where physical therapy is beneficial.

A physical therapist accesses a patient’s needs and then caters an exercise program to their personal abilities. They incorporate stretches, endurance exercises, strength training, and balancing activities. Exercising in the presence of a trained professional can decrease the likelihood of injury and over-exertion. Those with early onset Parkinson’s are more successful in physical therapy.

Caring for A Loved One with Early Onset Parkinson’s Disease

Those with early-onset Parkinson’s disease often struggle with the restrictions of their illness. After all, nobody expects to get ill while young. The support of family and friends is essential to adapting to life with a chronic illness. As rewarding as caring for a loved one with early onset Parkinson’s disease, the task Those with early-onset Parkinson’s disease often struggle with the restrictions of their illness. After all, nobody expects to get ill while young. Support from family and friends is essential to adapting to life with a chronic illness. Caregivers assist in daily activities, facilitate medical appointments, and make financial decisions. The tasks of caring for a loved one diagnosed with early onset Parkinson’s does not have to be daunting. With these tips, you can happily surmount the unknowns together.

Learn

As a caregiver, you will encounter situations where you will have to advocate for your loved one’s needs. To advocate properly, you must be informed of what early onset Parkinson’s entails. Research the latest treatments, familiarize yourself with your loved one’s medical history, and learn what you can about the disease.

Support Groups

Support groups are not just for patients. They are available to family and caregivers too. Find a local support group. Speaking with others who understand makes the stressors of caregiving easier.

Watch For Depression

Depression in early onset Parkinson’s is typical, particularly as the patient is adapting to their new life. You are around your loved one more than anybody else when caring for them around the clock. Recognize the signs of depression to report to your loved one’s doctor: crying, persistent sadness, poor appetite or over–eating, loss of motivation and interest in hobbies, thoughts of death, and feeling restless.

Prevent Caregiver Burnout

Caregiver burnout is the physical, emotional, and mental exhaustion that is the result of placing your loved one’s needs above your own. A number of factors lead to caregiver burnout—ranging from role confusion to unrealistic expectations. You might feel guilty attending to your physical, mental, and spiritual health, but you cannot care for another unless you first care for yourself.

Preventing caregiver burnout is essential to preserving your health. Do something for YOU. Schedule breaks into your day. Even sporadic 15-minute increment breaks are enough to enjoy a cup of tea, meditate, pray, or exercise. Do not forget to eat. If you are overwhelmed, ask for help. Delegate responsibilities to other family members or hire a home health aide. I can almost guarantee your loved one with early onset Parkinson’s values and appreciates all you do.

References

Webb, J. & Willette, A.A. (2017). Aging modifies the effect of GCH1 RS11158026 on DAT uptake and Parkinson’s disease clinical severity. Neurobiology of Aging, 2017; 50: 39 DOI: 10.1016/j.neurobiolaging.2016.10.006

Cheyanne Perry

Cheyanne is currently studying psychology at North Greenville University. As an avid patient advocate living with Ehlers Danlos Syndrome, she is interested in the biological processes that connect physical illness and mental health. In her spare time, she enjoys immersing herself in a good book, creating for her Etsy shop, or writing for her own blog.