Parkinson's (Parkinson's Disease, Parkinson-plus Syndromes). Causes

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)

Professional care

Ceballos-Baumann

Prof. Dr. med.

Andres Ceballos-Baumann


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