Q) My dad was recently diagnosed with something called Parkinsonism. I’ve never heard of this. How does it differ from Parkinson’s disease?
A) Most of us have heard of Parkinson’s, which while uncommon (1-2 people per 1000 at any age, rising in frequency to approximately 1% of the population 60 years of age and older) is still far more common than Parkinsonism. To put that in terms of raw numbers, it is estimated that there are about 1 million Americans diagnosed with Parkinson’s and no more than 10,000 who have Parkinsonism.
Now, from a medical term standpoint, this is not quite technically correct. Parkinsonism is not really a diagnosis in and of itself. Rather it refers to a set of symptoms which include tremor, stiffness, slowness of movement and challenges with walking and balance. As such, Parkinson’s disease is just one of 7 that share this set of symptoms (more or less) with other disorders such as; drug-induced parkinsonism, vascular parkinsonism (VP), multiple system atrophy (MSA), dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP) and corticobasal ganglionic degeneration (CBGD).
Collectively, these other six conditions are known as atypical Parkinson’s (AP) or Parkinsonism to most people. The fact that these seven conditions share the same set of symptoms (although people with AP often do not have the tell-tale hand tremor of true Parkinson’s) listed above probably reflects the fact that all of them seem to be associated with the loss of brain cells in the substantia nigra region of the brain.
However, people affected by the atypicals also seem to have much more widespread degradation of dopamine receptors (dopamine is the neurotransmitter that is most involved with movement and its relative absence is the foundation of Parkinson’s and many other movement disorders) in the striatum part of our nervous system.
This results in these people also having additional symptoms on top of the classic Parkinson type ones listed above which is one of the main ways clinicians distinguish between the different disorders. Unfortunately, another sign that you may have more than just typical Parkinson’s is if the condition seems to progress far more rapidly. For many of these atypical disorders, the average lifespan is 5 to 10 years after diagnosis with the level of disability reaching substantial proportions within a few short years.
Still, initially at least, all these disorders look the same and given the relatively far greater frequency of classic Parkinson’s, it is not uncommon for these disorders to be misdiagnosed for their more famous sister disease state.
The drug of choice for Parkinson’s is Sinemet (levodopa-carbidopa) or a class of drugs known as the dopamine agonists. In PD, patients will experience a noticeable improvement often within one day of starting Sinemet or a similar drug. Regrettably, people affected by the AP’s do not tend to respond to these drugs and their failure to do so should serve as a “red-flag” to the doctor that the individual may need a different diagnosis.
The actual “pinning” down of such is hard however as there is no single specific test that can be performed to easily distinguish these conditions from each other. Not that this is terribly critical for treatment as there are no known cures for any of the six atypicals and instead therapy should focus on physical therapy, occupational therapy and, perhaps most critically, exercise.
I thought I would end up this column by giving an extremely brief synopsis of each of the six different disorders that make up the AP’s.
Probably the best known of the lot is vascular parkinsonism (VP). This is due to the widespread press coverage it received when President George Bush was diagnosed with it towards the end of his life. VP is thought to occur as a result of a series of small strokes in the part of the brain that controls movement. The most prominent feature of this disorder is a gait abnormality (i.e. abnormal walking) and while traditional Parkinson’s drugs often don’t work they are still often trialled as some do respond and regain some degree of their lost mobility.
Progressive supranuclear palsy (PSP) is the most commonly diagnosed of the AP’s. In PSP, which has a number of different subtypes, the key early symptom is frequent falls, particularly in the backwards direction. As time goes on, patients often lose the ability to move their eyes voluntarily and their faces have prominent facial folds making them appear to be constantly worried or in an anxious state. In much later stages, difficulties with swallowing are a major concern.
In corticobasal ganglionic degeneration (CBGD), the disease is usually asymmetric meaning that one side of the body is far more affected than the other. It’s usually manifested by an arm that twitches chronically and also displays brief, lightning-like involuntary jerks. In multiple system atrophy, the Parkinson’s movement type features are often accompanied by urinary challenges and problems controlling ones blood pressure. Later on, it is common to see tilting of the torso, flexion of the neck and dysfunction of the voice and swallowing reflex.
In dementia with Lewy body the prominent associated symptoms beyond the typical ones are hallucinations and, not surprisingly given the name, an early onset of dementia.
The last type we’ll discuss is drug-induced parkinsonism. This occurs because there are a number of medications that “block” dopamine receptors within the brain causing the individual to act like they lack dopamine, such as is the case with Parkinson’s and the other atypicals.
Drugs that may do this (but certainly not in everyone who takes them) include multiple antipsychotics (such as olanzapine, aripiprazole & most of their sister drugs), drugs used to treat nausea (metoclopramide & prochlorperazine…), decongestants (including pseudoephedrine & phenylephrine which are found in many non-prescription cold remedies), pain killers (most notably tramadol), herbs (e.g. St. John’s Wort) and drugs used to reduce blood pressure (such as diltiazem and methyldopa). This is far from a complete list and the primary treatment here is to wean the individual off of the possibly offending agent and replace it with an alternative that does not impact the dopaminergic system.
In general, there is almost no reason for an individual with Parkinson’s (or an atypical) or at risk for it (such as those of us with a parent diagnosed with Parkinson’s at an early age) to ever be on any of these drugs since there are so many other, safer in their particular cases, options for each of them.