Parkinson’s Syndrome: Symptoms, Progression, Treatment

Brief overview

Symptoms: Slowed movements, lack of movement, muscle stiffness, tremors at rest, lack of stability of upright posture, rigid facial expression
Course and prognosis: Progressive, incurable disease; prognosis depends on course; with optimal treatment, life expectancy is often normal
Causes: Death of dopamine-producing cells in the brain; often unknown causes, some are caused by drugs and toxins or genetic changes
Examinations: Physical and neurological examination, L-dopa test, computed tomography (CT), magnetic resonance imaging (MRI)
Treatment: medication (such as levodopa), physiotherapy, speech therapy, occupational therapy, deep brain stimulation (THS)

What is Parkinson’s?

Parkinson’s disease, also called Parkinson’s disease, Parkinson’s disease or – colloquially – shaking paralysis, is one of the most common diseases of the central nervous system. In this progressive, neurodegenerative disease, certain nerve cells in the brain that produce the neurotransmitter dopamine die.

Men and women are affected by Parkinson’s disease at about the same rate. The average age at the time of diagnosis is around 60. Only about ten percent of those affected develop the disease before the age of 40.

What are the symptoms of Parkinson’s?

Parkinson’s syndrome usually develops gradually. In many people, the disease is initially heralded by non-specific early symptoms before the typical movement disorders appear.

Parkinson’s symptoms in the early stages

Signs of the progressive brain disease appear in some people years before the main symptoms:

Affected people talk, laugh or gesticulate while dreaming because these activities are not blocked in them during dream sleep (REM sleep behavior disorder), as is the case in healthy people.
The sense of smell is diminished or fails completely (hyposmia/anosmia).
There are sensory disturbances and pain in muscles and joints, especially in the neck, back and extremities.
Activities such as standing up, washing or dressing take longer than before.
Handwriting seems cramped and becomes smaller, especially at the end of a line or page.
Affected persons feel stiff, shaky and unsteady.
Facial expressions decrease and the face loses its expression.
Affected persons are often tired and fatigued and move little.
Changes in emotional life occur. For example, those affected are depressed or irritable for no particular reason, withdraw socially and neglect their hobbies.

Many of these early Parkinson’s symptoms are very non-specific. So many other causes are possible, such as advanced age. That’s why they are often not recognized as early signs of Parkinson’s.

The most important early sign is REM sleep behavior disorder: those who exhibit this form of sleep disturbance are generally at increased risk for neurodegenerative diseases. These are progressive diseases that involve the loss of nerve cells. Most people with REM sleep behavior disorder later develop Parkinson’s disease. Others develop a specific form of dementia (Lewy body dementia).

Main symptoms (cardinal symptoms) in Parkinson’s disease

In the advanced stage of Parkinson’s disease, the typical movement disorders come to the fore. Relatives and friends often notice these earlier than the affected person himself. In most cases, the Parkinson’s symptoms begin on one side, i.e. on only one side of the body. Later, they spread to the other side as well. In the course of the disease, they also become more pronounced.

The typical Parkinson’s symptoms are:

Affected persons are often tired and fatigued and move little.
Changes in emotional life occur. For example, those affected are depressed or irritable for no particular reason, withdraw socially and neglect their hobbies.

Many of these early Parkinson’s symptoms are very non-specific. So many other causes are possible, such as advanced age. That’s why they are often not recognized as early signs of Parkinson’s.

The most important early sign is REM sleep behavior disorder: those who exhibit this form of sleep disturbance are generally at increased risk for neurodegenerative diseases. These are progressive diseases that involve the loss of nerve cells. Most people with REM sleep behavior disorder later develop Parkinson’s disease. Others develop a specific form of dementia (Lewy body dementia).

Main symptoms (cardinal symptoms) in Parkinson’s disease

The typical Parkinson’s symptoms are:

Muscle stiffness can be detected by the so-called cogwheel phenomenon: When the doctor tries to move the affected person’s arm, the stiff muscles prevent a fluid movement. Instead, the arm can only be moved a little at a time and in a jerky manner. It almost feels like there is a cog in the joint that allows movement only to the next notch at a time and then locks into place.

Muscle tremor at rest (resting tremor).

In Parkinson’s disease, arms and legs typically begin to tremble in resting situations. This is why the disease is colloquially called “shaking paralysis.” One side of the body is usually more affected than the other. In addition, the arm usually trembles more than the leg.

Parkinson’s tremor typically occurs at rest. This makes it possible to distinguish Parkinson’s from other conditions involving tremors.

Lack of stability of upright posture

Unconsciously, every person corrects his or her posture at all times when walking or standing upright. The whole thing is controlled by so-called position and hold reflexes, i.e. automatic, involuntary movements that are triggered by certain stimuli.

Parkinson’s disease: accompanying symptoms

In addition to the main symptoms of Parkinson’s disease, some people experience other symptoms. However, these are not specific to Parkinson’s disease, but also occur with other diseases. These include, for example:

Psychological effects and personality changes such as listlessness, depression or anxiety.
Excessive sebum production of the facial skin, the skin appears greasy and shiny (ointment face)
Speech disorders (often soft, monotonous, blurred speech)
Eye movement disorders and eye tremor (eye tremor)
Swallowing disorders
Sleep disorders
In advanced disease, disturbances of the autonomic nervous system (for example, bladder weakness, constipation, erectile dysfunction, drop in blood pressure)

Parkinson’s dementia

People with Parkinson’s disease are more susceptible to dementia than the general population: approximately one-third of those affected also develop dementia during the course of the disease.

The symptoms of Parkinson’s dementia primarily include impaired attention and slowed thinking. This is an important difference from Alzheimer’s disease – the most common form of dementia. Alzheimer’s sufferers mainly from memory disorders. In Parkinson’s dementia, on the other hand, these only occur in the later stages of the disease.

You can read more about this topic in the article Dementia in Parkinson’s Disease.

As more and more nerve cells die over time in Parkinson’s syndrome, the disease progresses slowly, but not in relapses, as is the case with multiple sclerosis, for example. To date, Parkinson’s syndrome cannot be cured. All therapies alleviate the symptoms, but do not stop the destruction of the dopamine-producing nerve cells. Depending on the symptoms, doctors distinguish between four different forms of Parkinson’s disease:

Akinetic-rigid type: There is mainly immobility and muscle stiffness, while tremor is hardly or not at all present.
Tremor-dominant type: The main symptom is tremor.
Equivalence type: immobility, muscle stiffness, and tremor are approximately equal in severity.
Monosymptomatic resting tremor: Tremor at rest is the only symptom (very rare course).

The tremor dominance type has the most favorable prognosis: Although affected patients respond relatively poorly to therapy with L-dopa, this form progresses more slowly than the others.

In addition to the form of progression, the age of onset plays an important role in Parkinson’s disease. The course and prognosis depend on whether the disease breaks out at a relatively young age or at an older age. After about ten years of disease progression, life expectancy in Parkinson’s disease is slightly reduced.

Parkinson’s disease: life expectancy

According to statistics, an optimally treated person with Parkinson’s syndrome today has almost the same life expectancy as a healthy person of the same age. Today, if someone is diagnosed with Parkinson’s at the age of 63, it is estimated that they will live another 20 years. By comparison, in the middle of the last century, patients lived an average of just over nine years after diagnosis.

The increased life expectancy in idiopathic Parkinson’s syndrome is due to the fact that modern medications largely eliminate the main symptoms of those affected. In the past, such symptoms often led to complications and premature death. For example, Parkinson’s sufferers who were no longer able to move (akinesia) were often bedridden. This bedriddenness massively increases the risk of dangerous diseases such as thrombosis or pneumonia.

The improved life expectancy described here refers only to idiopathic Parkinson’s syndrome (= “classic Parkinson’s”). Atypical Parkinson’s syndromes, in which the affected person does not respond or hardly responds to treatment with L-dopa, usually progress more rapidly. They usually have a significantly worse prognosis.

Driving with Parkinson’s?

Therefore, it is necessary for affected individuals to have their driving ability evaluated by a physician or psychologist every 4 years.

What are the causes of Parkinson’s disease?

Medical professionals also call Parkinson’s disease primary or idiopathic Parkinson’s syndrome (IPS). “Idiopathic” means there is no tangible cause for the disease. This “true” Parkinson’s accounts for about 75 percent of all Parkinson’s syndromes. To be distinguished from this are the rare genetic forms of Parkinson’s, “secondary Parkinson’s” and “atypical Parkinson’s.”

Idiopathic Parkinson’s: Dopamine Deficiency

Parkinson’s disease originates in a specific brain region called the “black matter” (substantia nigra) in the midbrain. The substantia nigra contains special nerve cells that produce the nerve messenger (neurotransmitter) dopamine. Dopamine is very important for the control of movements.

As cell death progresses, the level of dopamine in the brain continues to drop – a dopamine deficiency develops. The body compensates for this for a long time: Only when about 60 percent of the dopamine-producing nerve cells have died does the dopamine deficiency become noticeable in the form of the movement disorders typical of Parkinson’s disease.

But the dopamine deficiency itself is not the sole cause of Parkinson’s: it also disturbs the delicate balance of nerve messengers. Because less and less dopamine is available, the amount of the neurotransmitter acetylcholine, for example, increases. Experts suspect that this is the reason for the tremor and muscle stiffness (rigor) in Parkinson’s disease.

The imbalance of neurotransmitters in Parkinson’s may also be the reason why many sufferers also become depressed. However, the link between Parkinson’s disease and depression has not yet been conclusively established.

Genetic forms of Parkinson’s disease

If a family member has Parkinson’s disease, this unsettles many relatives. They wonder whether Parkinson’s is hereditary. In the vast majority of cases, however, Parkinson’s is the idiopathic Parkinson’s described above. Experts believe that heredity plays no role in this sporadic form of the disease.

Secondary Parkinson’s syndrome

Unlike idiopathic Parkinson’s, symptomatic (or secondary) Parkinson’s syndrome has clearly identifiable causes. Some important triggers and risk factors include:

Medications that inhibit dopamine (dopamine antagonists), such as neuroleptics (used to treat psychosis) or metoclopramide (used to treat nausea and vomiting), lithium (used to treat depression), valproic acid (used to treat seizures), calcium antagonists (used to treat high blood pressure)
Other diseases such as brain tumors, inflammation of the brain (e.g., as a result of AIDS), parathyroid hypofunction (hypoparathyroidism), or Wilson’s disease (copper storage disease)
poisoning, for example with manganese or carbon monoxide
Injuries to the brain

The influence of alcohol on the risk of developing Parkinson’s disease has not been conclusively determined. However, current study data indicate that there is probably no connection. There may be an increased risk with excessive alcohol consumption.

Atypical Parkinson’s syndrome

Neurodegenerative diseases, some of which cause atypical Parkinson’s syndrome , include:

Lewy body dementia
Multisystem atrophy (MSA)
Progressive supranuclear palsy (PSP)
Corticobasal degeneration

Such disorders have a much worse prognosis than “true” (idiopathic) Parkinson’s disease.

The drug L-dopa, which works very well in idiopathic PD, is of little or no help in atypical PD.

Investigations and diagnosis

It is still often difficult to diagnose Parkinson’s without doubt. One reason for this is that there are many different diseases that cause symptoms similar to those of Parkinson’s disease.

The doctor-patient interview (anamnesis interview) and the physical-neurological examination are indispensable for the diagnosis of Parkinson’s disease. The further examinations serve primarily to exclude other causes for the symptoms. Only if the symptoms can be well explained by Parkinson’s and the doctor finds no other causes, it is an idiopathic Parkinson’s syndrome.

The correct contact person when Parkinson’s syndrome is suspected is the neurologist, i.e. a specialist in diseases of the nervous system.

Medical history

How long has the tremor of the hands/legs existed?
Does the affected person have the feeling that the muscles are constantly tense?
Does the affected person have pain, for example in the shoulder or neck area?
Does the affected person find it difficult to maintain balance when walking?
Do fine motor activities (e.g. buttoning a shirt, writing) become increasingly difficult?
Are there problems sleeping?
Has the sense of smell deteriorated?
Has a relative been diagnosed with Parkinson’s disease?
Is the person affected taking medication, for example due to psychological problems?

Physical and neurological examination

After the anamnesis interview, a physical and a neurological examination follow. During this examination, the doctor generally checks the function of the nervous system: for example, he tests the reflexes, the sensitivity of the skin and the mobility of the muscles and joints.

He pays particular attention to the main symptoms of Parkinson’s, such as slowed movements, an unsteady gait or noticeable gestures and facial expressions. The doctor also detects the tremor at rest (resting tremor) typical of Parkinson’s during the physical examination.

Various tests help the doctor to confirm the Parkinson’s diagnosis. These include the following tests:

Pendulum test: In this test, the doctor shakes the arms of the affected person. in people with Parkinson’s, the pendulum movement is reduced.
Wartenberg test: The doctor lifts the head of the affected person in the supine position and then suddenly releases it. In people with Parkinson’s, it falls back slowly or not at all.

Parkinson’s test (L-dopa test and apomorphine test).

To support the Parkinson’s diagnosis, the doctor sometimes performs the so-called L-dopa test or an apomorphine test. In this test, affected persons are given the dopamine precursor L-dopa or apomorphine once, i.e. two drugs that are used in therapy. In Parkinson’s syndrome, symptoms sometimes improve shortly after taking the drugs.

However, both tests are of limited value in Parkinson’s diagnostics. This is because some people have Parkinson’s but do not respond to the tests. Conversely, the L-dopa test is sometimes positive in other diseases. Because of these problems, neither test is routinely used in Parkinson’s diagnosis.

Imaging techniques

Computed tomography (CT) or magnetic resonance imaging (MRI) can be used to image the brain. This helps the doctor rule out other possible causes for the symptoms, such as a brain tumor.

Less complex and less expensive is the ultrasound examination of the brain (transcranial sonography, TCS). It helps to detect idiopathic Parkinson’s syndrome at an early stage and to distinguish it from other diseases (such as atypical Parkinson’s syndromes). However, the physician should have extensive experience with this examination in order to interpret the results correctly.

Special case: Genetic Parkinson’s disease

The rare genetic forms of Parkinson’s disease can be detected by molecular genetic testing. Such testing may be considered if:

the affected person develops Parkinson’s disease before the age of 45, or
at least two first-degree relatives have Parkinson’s disease.

In these cases, it is suspected that the Parkinson’s disease is caused by a genetic mutation.

Treatment

There is currently no cure for Parkinson’s disease. However, the symptoms can be significantly alleviated and the quality of life improved by various treatment options. The doctor adapts the Parkinson’s therapy individually to each person affected. This is because the symptoms vary from person to person and progress at different rates.

Individual Parkinson’s treatment often includes other components. These include, for example, physiotherapy, speech therapy and occupational therapy. In any case, it makes sense to seek treatment in a special Parkinson’s clinic.

Parkinson therapy with medication

There are various medications for Parkinson’s therapy. They help against symptoms such as slowed movements, rigid muscles and tremors. However, they do not prevent the dopamine-producing nerve cells from dying and the disease from progressing.

The typical Parkinson’s symptoms are triggered by a lack of dopamine in the brain. They can therefore be alleviated either by supplying the neurotransmitter as a drug (for example, in the form of L-dopa) or by preventing the breakdown of the dopamine that is present (MAO-B inhibitors, COMT inhibitors). Both mechanisms compensate for the dopamine deficiency. They thus largely eliminate the typical Parkinson’s symptoms.

L-dopa (levodopa)

L-dopa is very effective and has hardly any side effects. Doctors prescribe it mainly for people over the age of 70. In younger patients, on the other hand, they use L-dopa only very cautiously. The reason is that treatment with L-dopa sometimes triggers movement disorders (dyskinesias) and fluctuations in effect after a few years.

Fluctuations in the effect of L-dopa

Long-term treatment with L-dopa sometimes causes the effect of the drug to fluctuate (fluctuations in effect): Sometimes it is no longer possible for those affected to move at all (“OFF phase”), then it works completely normally again (“ON phase”).

In such cases, it helps to change the dosage or to switch to a retarded L-dopa preparation: Retard tablets release the active ingredient more slowly and over a longer period than “normal” (unretarded) L-dopa preparations.

A drug pump is also helpful in case of fluctuations in the effect of L-dopa (ON-OFF phases) and/or movement disorders. It automatically delivers the levodopa via a thin tube directly into the duodenum, where it is absorbed into the blood. The patient thus receives the active ingredient continuously, resulting in very uniform levels of active ingredient in the blood. This reduces the risk of fluctuations in effect and movement disorders.

Dopamine agonists

In patients younger than 70, the doctor usually starts Parkinson’s therapy with dopamine agonists. This can delay the onset of movement disorders such as those triggered by the prolonged use of L-dopa.

With prolonged use, dopamine agonists also sometimes trigger fluctuations in effect. However, this happens much less frequently than with L-dopa. The fluctuating effect can be compensated for by the doctor adjusting the dosage, prescribing a different preparation or using a drug pump.

MAO-B inhibitors

MAO-B inhibitors inhibit the enzyme mono-amino oxidase-B (MAO-B), which normally breaks down dopamine. In this way, dopamine levels can be increased in the brains of people with Parkinson’s disease. MAO-B inhibitors are less effective than levodopa or dopamine agonists. As a stand-alone Parkinson’s therapy, they are therefore only suitable for mild symptoms (usually in the early stages of the disease). However, they can be combined with other Parkinson’s medications (such as L-dopa).

COMT inhibitors

COMT inhibitors are prescribed together with L-dopa. They also block an enzyme that breaks down dopamine (called catechol-O-methyl transferase = COMT). In this way, COMT inhibitors prolong the effect of dopamine. These drugs are prescribed primarily to reduce fluctuations in effect (fluctuations) during therapy with L-dopa. Thus, they are medications for advanced stages of Parkinson’s disease.

Anticholinergics

The so-called anticholinergics were the first drugs that doctors used for Parkinson’s therapy. Today, they are not prescribed as often.

NMDA antagonists

Like acetylcholine, the neurotransmitter glutamate is also present in Parkinson’s in relative excess due to dopamine deficiency. So-called NMDA antagonists help to counteract this. They block certain docking sites of glutamate in the brain and thus reduce its effect. The doctor prescribes NMDA antagonists primarily in the early stages of Parkinson’s syndrome.

In rare cases, a change in medication or acute concomitant diseases can lead to an akinetic crisis. This is a sudden worsening of symptoms with total immobility. Affected persons are also no longer able to speak or swallow. The akinetic crisis is an emergency and requires immediate treatment in the hospital.

The medications used for Parkinson’s sometimes cause psychosis. It is estimated that up to 30 percent of all people with the disease experience such a crisis. It is initially manifested by restless sleep with vivid dreams, and later also by hallucinations, delusions and states of confusion. It is important in this case to consult a neurologist immediately.

Deep brain stimulation

Thus, deep brain stimulation works similarly to a cardiac pacemaker. It is therefore sometimes referred to as a “brain pacemaker” (although this term is not entirely correct).

Deep brain stimulation is considered when:

fluctuations in action (fluctuations) and involuntary movements (dyskinesias) cannot be alleviated with medication, or
tremor (tremor) does not respond to medication.

In addition, the affected person must meet other requirements. For example, he must not show early symptoms of dementia. His general physical condition must be good. In addition, the Parkinson’s symptoms (except tremor) must respond to L-dopa.

Experience has shown that the intervention effectively alleviates symptoms in many affected individuals and significantly improves quality of life. This effect also appears to last over the long term. However, this does not mean that deep brain stimulation cures Parkinson’s disease: The disease progresses even after the procedure.

Originally, deep brain stimulation was used primarily for advanced Parkinson’s. However, studies now show that it is also well suited to sufferers under the age of 60, in whom L-dopa therapy has only recently begun to show fluctuations in effect and cause movement disorders.

Possible complications and side effects of deep brain stimulation

The most important complication of brain surgery is bleeding in the skull (intracranial bleeding). In addition, the insertion of the pulse generator and cables sometimes triggers an infection. Then it is necessary to temporarily remove the system and treat the infection with antibiotics.

Almost every affected person experiences temporary side effects after the procedure. These include, for example, paraesthesia. However, these often only occur immediately after the pulse generator is switched on and then disappear again.

Other usually temporary effects are, for example, confusion, increased drive, flattened mood and apathy. Sometimes so-called impulse control disorders also occur. These include, for example, increased sexual desire (hypersexuality). In some people, deep brain stimulation also triggers mild speech disorders, a disturbance in movement coordination (ataxia), dizziness, and unsteadiness in walking and standing.

Other therapy methods

Various treatment concepts additionally help people with Parkinson’s syndrome to maintain their mobility, ability to speak and independence in everyday life for as long as possible. The most important methods are:

Physical therapy (physiotherapy), for example, to improve balance and safety when walking and to improve strength and speed
Occupational therapy to better manage everyday life with the disease and remain independent in the personal environment for as long as possible (For example, the therapist shows the affected person how to use certain aids and, together with the affected person, adapts the living space so that he or she can find his or her way around better).

Parkinson’s disease: treatment in specialized clinics

People with Parkinson’s syndrome should be treated in a specialized clinic if possible. Doctors and other employees there are specialized in the disease.

There are now numerous clinics in Germany that offer acute treatment and/or rehabilitation for people with Parkinson’s disease. Some of these carry a certificate from the German Parkinson’s Association (dPV). It is awarded to hospitals and rehabilitation facilities that have special diagnostic and therapeutic services for people with Parkinson’s disease and related disorders.

For a selected list of specialty clinics for people with Parkinson’s, see the article Parkinson – Clinic.

Living with Parkinson’s: What can you do yourself?

In addition to medical treatment, the behavior of the person with Parkinson’s itself potentially contributes a great deal to effective therapy:

Inform yourself about the disease. Because in many cases, the fear of the unknown is particularly stressful. The more those affected learn about the disease, the sooner the feeling of powerlessness in the face of the progressive disease disappears. Even as a relative of a Parkinson’s patient, you should inform yourself about the disease in order to support your relative effectively and meaningfully.

Join a Parkinson’s support group. Those who have the opportunity to regularly exchange ideas with others affected often cope better with the disease.

Stay fit. Try to maintain a good general condition by staying physically active. Regular exercise (such as walks) and light endurance sports are sufficient.

Use small aids in everyday life. Many Parkinson’s symptoms make everyday life more difficult. These include “freezing” – when the person is no longer able to move. Visual stimuli on the floor, such as footprints stuck on the floor, or acoustic rhythms (“left, two, three, four”) can help. Important for fellow patients: There is no point in urging or pulling the affected person to hurry. This tends to prolong the “freezing” episode.

Prevention

Since the causes of idiopathic Parkinson’s syndrome are unknown, there are no specific measures to prevent the disease.

Further Information

Book recommendations:

Parkinson’s – the exercise book: Staying active with movement exercises (Elmar Trutt, 2017, TRIAS).
Parkinson’s disease: A guidebook for those affected and their relatives (Willibald Gerschlager, 2017, Facultas / Maudrich)

Self-help groups:

Deutsche Parkinson Vereinigung e. V.: https://www.parkinson-vereinigung.de

Deutsche Parkinson Hilfe e. V.:https://www.deutsche-parkinson-hilfe.de/