Parkinson's disease (Parkinson's syndrome).

Parkinson's disease - A 21st century disease.

According to the World Health Organisation, from 2040 onwards neuro-degenerative diseases like Parkinson's and Alzheimer's will overtake cancers as the second most common cause of death after cardiovascular diseases. In the next 20 years it is expected that there will be a doubling in the numbers of people with Parkinson's disease (idiopathic Parkinson's disease, primary Parkinsonism).

 

Parkinson's disease. Information from A-Z

Information. What is Parkinson's disease?

The name Parkinson's disease refers back to the London surgeon, palaeontologist and politician James Parkinson who in 1817 published a treatise entitled "An Essay on the Shaking Palsy" which for the first time described the clinical picture of six patients whom he had observed in the street with what came to be called "Parkinson's disease". Parkinson was the first person to trace the various symptoms back to a common cause in the brain. The Latin name used to describe this condition, paralysis agitans, or agitated paralysis, later became known colloquially as the "shaking palsy". The term palsy technically means a loss of motor function or paralysis. This is somewhat misleading because this condition does not involve paralysis and the primary symptom is the tremor or shaking which the patient experiences. A tremor features in only about 50% of patients with Parkinson's disease and is found more frequently in people who do not have Parkinson's, in people who have another important type of motor disorder, the so-called essential tremor.

 

Parkinson´s disease.

Parkinson's disease, which is also called primary or idiopathic Parkinsonism, in the narrower sense, is the prototypical form of the other Parkinson-plus syndromes. The modern anti-Parkinson's medications are really licensed for use in treating this from of the disease. Modern diagnostic criteria are used to distinguish primary Parkinsonism from other Parkinson-plus syndromes. Parkinson's disease or primary Parkinsonism in this narrower sense is defined by the presence of particular changes in the nervous system as well as by the symptoms and course of the disease and by the patient's response to dopamine replacement medications (L-DOPA, dopamine agonists, MAO-B inhibitors and COMT inhibitors): The disease is characterised by the premature death (degeneration) of certain types of neurons or nerve cells in the brain. The most marked deterioration of these neurons is found in an area of the brain called the substantia nigra (or "black substance": This is an area of gland-like dopamine-producing structures in the brain stem). This is the brain's primary site for the production of the neurotransmitter dopamine. Another feature of Parkinson's disease, which is also present in other degenerative diseases affecting the brain and nervous system (neurodegenerative diseases) are aggregates of protein which develop inside the nerve cells. These were discovered in 1912 by Friedrich Levy (later known as Frederic Lewy) and are called Lewy bodies.

 

What are Parkinson's disease and Parkinson-plus syndromes?

Parkinson's syndrome has a similar clinical manifestation to Parkinson's disease.

 

Definition of Parkinson-plus syndromes.

A case of Parkinson's syndrome, also known as Parkinsonism, secondary or atypical Parkinson's, when separated from its cause, is defined primarily by:

  • Slowness of physical movement (bradykinesia),
  • Loss of spontaneous movements such as gestures, a "masked" facial expression (a look of surprise with raised eyebrows), Exaggerated swinging of the arms while walking (hypokinesia)
  • Difficulty in initiating movement (akinesia).

The three concepts of akinesia, bradykinesia and hypokinesia are used interchangeably in medical terminology. One of the following three symptoms must also be present:

  • Muscle stiffness (rigor)
  • Tremor when the body is at rest
  • Balance disorders (Disorder of the postural reflexes)
 

What is an akinetic crisis?

An akinetic crisis is understood in terms of an acute deterioration in the symptoms of Parkinson's with akinesia (lack of movement), swallowing problems and the frequently accompanying symptoms of raised blood pressure and sweating. The triggering factors are usually withdrawal from medication and/or concomitant illnesses (e.g. pneumonia or surgical intervention). This situation indicates an emergency.

 

Symptoms. Signs of Parkinson's disease

Up to 50% of humanity is affected by Parkinsonian symptoms

Up to 50% of humanity is affected by Parkinsonian symptoms

Even more common than Parkinson's disease itself, in the narrower sense, by which about 300,000 people in Germany are affected, are people who have Parkinsonian symptoms. Parkinsonian symptoms are found especially amongst people with all types of neuro-degenerative diseases. Whereas 10% of people over the age of 65 have at least two Parkinsonian symptoms, around 50% of people over the age of 85 will display some of these symptoms.

 

Parkinsonism Syndrome in Cerebrovascular insufficiency and so-called Normal Pressure Hydrocephalus.

Parkinson's syndrome is very common in patients with cerebral circulation disorders and with normal pressure hydrocephalus (enlarged brain ventricles-NPH). The primary feature of this disorder in its early stages is a movement disorder which particularly affects walking. The patient will characteristically walk in small shuffling steps, but unlike those of patients with classical Parkinson's disease the steps are broad based and are marked by a pronounced inability to initiate the steps and by sudden stopping (festination or "freezing" as if the patient is stuck to the ground). Unlike the symptoms found in classical Parkinson's disease, the movement of the upper body half, such as the facial expression, gestures and the swinging of the arms while walking, as well as the position of the posterior, are generally normal. They may even be exaggerated, (such as "paddling with the arms" while walking). In the USA the term "lower body Parkinson's” was coined to describe the Parkinsonism condition with this range of symptoms. Bladder problems, especially the urge to urinate are very pronounced here. These Parkinsonism syndromes are often treatable, either by repeated collection of cerebrospinal fluid from the lumbar spinal area or by the insertion of a special valve which regulates the pressure in the cerebral ventricles. It is important that any problems affecting the blood vessels are treated. With patients suffering from Parkinson's disease, in the narrower sense, great care must be taken to avoid the risks of cerebrovascular disorders (or insufficiency) from arising. These can all too often occur and if they do it can greatly complicate any form of Parkinsonism which is related to the cerebral circulation system.

 

Drug-induced Parkinsonism syndrome.

Certain types of medication may cause a particular syndrome of drug-inuced Parkinsonism. Typically these are psycho-active drugs, the classic neuroleptics, which work directly on the brain to suppress hallucinations and delusions. These drugs, are however, also used in the treatment of other mental health complaints. Especially when prescribing anti-Parkinsonism medications for older patients, the physician must specifically ask them whether or not they are taking any other forms of medication which may appear harmless but which work in a similar way to neuroleptics such as certain types of anti nausea drugs such as metoclopramide. Metoclopramide is in the final analysis a neuroleptic drug and can cause Parkinsonism in otherwise healthy patients. The anti nausea drug metoclopramide should therefore not be used for patients with Parkinson's disease. Alternative drugs such as domperidone should be used instead.

 

Multiple System Atrophy (MSA).

Today we understand MSA as a combination of symptoms and indicators of disease as found in Parkinson's disease and disorders of the vegetative nervous system (e.g. blood pressure regulation and bladder control) and the cerebellum.

A diagnostic pointer is the question about impotency in men, which can be a precursor many years prior to a diagnosis of MSA or in the case of both sexes urinary incontinence. Other important symptoms relate to

  • disturbances of circulatory regulation (e.g. pronounced feelings of dizziness and/or bouts of fainting (syncopes) when standing up
  • abnormal shaking (tremor when resting, holding something and/or action tremor), up to 60% of patients  
  •   abnormal postures (dystonias), in 46% of cases, e.g. in 25% of patients a strong forward flexion of the neck (anterocollis)
  •   very vivid dreams even violent sleep disturbances (in 90 % of patients), the so-called REM sleep behaviour disorders, which can precede the disease by years

Generally patients do not respond to long-term administration of dopamine substitutes such as l-dopa and dopamine. In the early stages however up to 30% of patients react to drugs for Parkinson's and in 10% a positive reaction to this medication is maintained even in the advanced stages. Therefore it is recommended that investigations into the response to dopamine substitutes &- in this case l-dopa be as standardised as possible.

 

Lewy body dementia.

Over time and even in the early stages Lewy body dementia patients develop a Parkinson's Plus Syndrome Classic physical symptoms of disease besides dementia are Parkinsonian symptoms, furthermore unexplained falls and hypersensitivity to neuroleptic drugs, which on administration of such drugs is expressed in the rapid onset of stiffness (rigour) and impaired movement (akinesia), drowsiness and confusion. The Lewy body form of dementia is typically characterised by strong variations in awareness or alertness and well formed and detailed illusionary perception (visual hallucinations). Unlike Alzheimer's disease Lewy body dementia patients are more apathetic and disorientation (place-related and spatial) is a more pronounced phenomenon than memory problems. Compared to Alzheimer's disease memory disorders in the early stages of Lewy body dementia are if anything discrete, in the early stages of Lewy body dementia there are characteristically strong variations in attention and concentration compared to Alzheimer's, which sometimes leads to the wrong diagnosis of a seizure disorder.

 

Progressive supranuclear palsy.

Progressive supranuclear palsy (abbreviated PSP), is a disease frequently described as a Parkinson's Plus Syndrome and is characterised by a restriction or paralysis in upward and downward conjugate eye movements. However the palsy associated with conjugate eye movements upwards or downwards (vertical gaze palsy - hence the name) only occurs in the later stages of the disease. Patients are initially noticeable because of their tendency to fall and tumble backwards. In addition there are other symptoms going beyond the typical symptoms of Parkinson's disease such as distinctive abnormalities in the voice and speech. Dementia is problematic and unlike actual Parkinson's disease has already developed in the early stages and after only a few years of disease progression. Dementia in the case of PSP differs from Alzheimer's disease. Recognition capabilities, unlike in Alzheimer's, remains intact for an astonishingly long time with PSP (similar to Lewy body dementia). With PSP thought processes are slowed down (bradyphrenia), and there are attention deficits and apathy ("lack of enthusiasm", loss of initiative and interest), which are problematic.

 

Corticobasal Degeneration.

The classic syndrome begins insidiously at around the age of 60 and in many patients is similar to typical idiopathic Parkinson's syndrome with marked side asymmetry of symptoms. Over time there are cramps and abnormal body postures (dystonias), muscle twitches (myclonias) and a freezing of gait and unsteadiness while standing. In addition there are strange feeling of alienation from parts of the body (the Alien Limb Phenomenon) and in the later stages abnormalities in the voice and speech together with dementia.

 

Other Parkinsonian Syndromes with Dementia.

Unlike Lewy body dementia in which a Parkinsonian syndrome belongs to the diagnostic criteria in the early stages of the disease, in the case of Alzheimer's disease a Parkinsonian syndrome only occurs in the advanced stages. A Parkinsonian syndrome may also be a predominant factor in Pick's disease, (frontotemporal lobe- degeneration - frontotemporal dementia). Wilson's disease is very important because its causes are treatable. The neurological symptoms caused by a disturbance in copper metabolism manifest themselves mostly in a Parkinsonian syndrome and neuropsychiatric changes such as a dementia type illness. In the case of Huntingdon's disease, especially in the rare Westphal variant beginning in early adulthood and the pantothenate kinase 2 (PANK2) associated neurodegenerations (NBIA syndrome - Neurodegeneration with Brain Iron Accumulation_ - nerve degeneration with the accumulation of iron in the brain, and also neuroaxonal dystrophy, formerly Hallervorden- Spatz disease, the disorder usually manifests itself from the very start in combination with a Parkinsonian syndrome with dementia.

 

Causes. What causes the Parkinson's disease?

How does the condition develop?

Although we know today that Parkinson's disease is caused by insufficient formation and action of dopamine in the brain and that, in the same way that we can administer insulin to diabetics to compensate for the body's inability to produce it in sufficient quantities, we can supply the necessary dopamine to people with Parkinson's in the form of dopamine substitutes (such as the dopamine precursor L-DOPA and synthetic substances which mimic the action of dopamine, the so-called dopamine agonists) we still do not understand the reason for the deterioration of "black substance" in the brain which is found in the classical from of Parkinson's disease even though it is now almost 200 years since the condition was first described by James Parkinson. It is likely that a single cause will never be found. Parkinson's disease must rather be viewed as being a complex clinical picture in which the disease progression is caused by an interplay of a particular predisposition to the condition and environmental factors. The discovery of certain rare inherited variants of the disease, which can be traced back to the presence of a single gene, has allowed researchers to make some important findings about the molecular basis for the cause of the disease. This allows us to believe that it will be possible in the not too far distant future to develop an effective form of neuroprotection (protection of the nerve cells which will slow down or even halt the progress of the disease). We cannot say with any certainty that Parkinson's disease is an inherited condition in the classical sense of the term. The development of therapeutically highly effective substitutes for the neurotransmitter dopamine containing levodopa which the body can convert into dopamine is closely associated with the discovery of dopamine as a central neurotransmitter and with its absence or shortage in the brain being a fundamental cause of Parkinson's disease. Arvid Carlsson was awarded the Nobel Prize for Medicine and Physiology in 2000 for his work on dopamine and Parkinson's disease.

 

Forms of Parkinsonism-plus syndromes.

The following different types or classes of Parkinsonism-plus syndromes are differentiated depending on their cause:

  • Parkinson's disease in the narrower sense: A good response to medications which contain dopamine replacement substances, such as levadopa and the dopamine agonists, is nowadays considered to be an essential clinical criterium for making a diagnosis of Parkinson's disease in the narrower sense.
  • Symptomatic (secondary) Parkinsonism and Parkinsonism-plus syndromes: Several other terms are used to describe these conditions: Parkinsonoid, Parkinsonism, Pseudo-Parkinsonism
  • The causes of secondary Parkinsonism syndromes
    The symptoms of Parkinsonism are caused by well-understood external influences, most commonly by disorders affecting the blood circulation in the brain (cerebral circulation) such as arteriosclerosis ("hardening of the arteries" = subcortical vascular encephalopathy). There are also many medications which can cause or worsen Parkinsonism. This is unavoidable.
  • Other causes are:
    - Normal pressure hydrocephalus (NPH), a specialised form of disorder affecting the cerebral ventricles.
    - Tumors
    - Post traumatic Parkinsonism
    - Carbon monoxide, manganese or potassium cyanide poisoning
    - Metabolic causes (e.g Wilson's disease, hypoparathyroidism, Fahr's disease)
 

Other forms of the condition: Atypical Parkinsonism syndromes, Parkinsonism-plus-syndromes.

The terms atypical Parkinsonism or Parkinsonism-plus syndromes are used to cover several more clinical pictures which up until now have proved somewhat more difficult to treat with medications than Parkinson's disease in the narrower sense. The term Parkinsonism-plus points to the presence in these conditions of additional clinical symptoms, such as dementia, incontinence, falling over and blood pressure irregularities which are not found in the classical form of Parkinson's disease, at least not in the first years of the condition. The course of these Parkinsonism-plus syndromes is generally faster and shorter than that of the classical form of the disease. In addition to the other forms of the condition affecting the blood vessels of the brain which have already been mentioned, the following conditions are the most important forms of Parkinsonism-plus syndromes.

  • Multiple System Atrophy (MSA)
  • Progressive Supranuclear Palsy (PSP). This condition is also known as
  • Steele-Richardson-Olszewski Syndrome(SROS).
  • Corticobasal Degeneration (CBD)
  • Lewy-Body Disease or Lewy-Body Dementia (LBD), also known as Dementia with Lewy Bodies (DLB)
 

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