In our first Post in this series we looked at the causes and symptoms of Parkinson’s Disease (PD). In this Post I would like to consider Parkinsonism, which is often confused with Parkinson’s Disease.

“Parkinsonism” is the clinical definition of a variety of different underlying pathologies that can cause Parkinson’s-like symptoms such as slowing of movement, tremor, rigidity or stiffness, and balance problems. There are a number of disorders that can produce the symptoms referred to as Parkinsonisms; Parkinson’s disease is just one of them. As such, Parkinson’s disease is actually a form of Parkinsonism. This is a more general term used to refer to the set of symptoms that is commonly associated with Parkinson’s disease but sometimes stems from other causes.

The distinction is important because some of these other causes may be treatable, while others do not respond to treatment or medication. The same symptoms that indicate Parkinson’s Disease can also indicate other conditions, thus Parkinsonism is a generic term referring to slowness and mobility problems that look like PD. Parkinsonism is a feature in several conditions that have different (and perhaps known) causes, but those conditions don’t progress like PD. As a result, years may go by before the differences between PD and the other disorders are apparent; the PD diagnosis may then be reversed.

Other causes of Parkinsonism include:

  • An adverse reaction to prescription drugs
  • Use of illegal drugs
  • Exposure to environmental toxins
  • Stroke
  • Thyroid and parathyroid disorders
  • Repeated head trauma (for example, the trauma associated with boxing and multiple concussions, which is believed to be the causative factor in Muhammad Ali’s PD)
  • Brain tumor
  • An excess of fluid around the brain (called hydrocephalus)
  • Brain inflammation (encephalitis) resulting from infection

Parkinsonism may also be present in persons with other neurological conditions, including Alzheimer’s, Lewy Body Dementia, Creutzfeldt-Jakob disease, Wilson’s disease, and Huntington’s disease.

So, what’s the difference between being diagnosed with Parkinson’s disease and having what are known as “Parkinsonisms”?  The typical Parkinson’s patient has Lewy bodies (aggregates of a protein called alpha-synuclein) in the brain’s neurons. When that patient is given dopamine replacement therapy those symptoms disappear.

By contrast, Parkinsonisms – also sometimes referred to as atypical Parkinson’s –have features in addition to typical Parkinson’s disease symptoms, and those symptoms do not respond to dopamine replacement therapy

Taking antiparkinsonian medications (such as levodopa) may be the first indicator that Parkinsonism isn’t actually PD. By definition, PD promptly responds to this medication and, while not a cure, dopamine enhancing medications improves its symptoms in a consistent way, at least for a few years. But in Parkinsonism, improvement is often erratic or nonexistent from the beginning.

Two categories of non-PD disorders are:

  • Parkinson’s Plus Syndromes: This group of neurodegenerative disorders has Parkinsonian features, such as bradykinesia (slowness), rigidity (stiffness), tremor (shaking), and gait disturbances (balance). However, they are also associated with other complex neurological symptoms that reflect problems in brain areas other than the dopaminergic system (the network of neurons able to make and release the neurotransmitter dopamine). These conditions progress more rapidly than PD and don’t respond as well (or at all) to antiparkinsonian medications. The most common Parkinson’s Plus syndromes are Multiple System Atrophy (MSA), Progressive Supranuclear Palsy (PSP), Cortico-Basal Ganglionic Degeneration (CBGD), and Lewy Body Dementia (LBD).
  • Secondary Parkinsonisms: The symptoms of these disorders relate to well-defined lesions in the brain from strokes, tumors, infections, traumas, or certain drugs. Like Parkinson’s Plus syndromes, these syndromes are usually less responsive to levodopa. However, if the primary cause of Parkinsonism is controlled, these symptoms tend to be less progressive.

In addition to Parkinson’s Plus and Secondary Parkinsonisms, Essential Tremor (ET) is another source of possible confusion. As the most common movement disorder — as much as 20 times more common than PD — ET’s only symptom is a tremor that affects the hands (only while they’re moving) but may also affect the head or voice. ET can run in families and is usually benign and non-disabling. The legendary actress, Katherine Hepburn, may have suffered from ET — not PD.

The diseases above are often grouped, or lumped, together to allow researchers to drill down to the molecular level and study what molecular links there might be between Parkinson’s disease and Parkinsonisms in hopes of finding a cure.

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