The Plague of Idiopathic
Parkinson’s Disease
Paul
and idiopathic Parkinson’s
Paul was a relatively healthy male up unto about ten years
ago when he began exhibiting troublesome symptoms. He started to lose his
ability to use his right arm properly. His movements became more difficult and
slow in the arm and led to him being moved to a desk job. His symptoms
gradually began to worsen until he was finally diagnosed with idiopathic
Parkinson’s disease. Before going too much further however, it is important to
have a little more background on Parkinson’s disease.
Sir James Parkinson was the first to discover Parkinson’s disease
which, in 1817, he referred to as “shaking palsy”. According to him, the
disease was “characterized by “involuntary tremulous motion, which lessened
muscular power, in part not in action and even when supported; with a
propensity to bend the trunk forward, and to pass from a walking to a running
place; the senses and intellect being uninjured.” (Ghiglione 2005).
Nowadays, Parkinson’s disease is described as being a degenerative disorder of
the central nervous system characterized by tremors, a shuffled gait and loss
of muscle movement. In the disease, the amount of the neuromodulator Dopamine
is decreased due to the degeneration of the substantia nigra. The lack of
dopamine in the system leads to the motor symptoms of Parkinson’s disease,
however it is not known as of yet what causes the symptoms to occur. There is
evidence to show that the disease may have a genetic factor. There are two
primary types of Parkinson’s disease: idiopathic and atypical Parkinson’s, both
of which will be explained further on ("Parkinson's disease:
Hope," 2013).
The first symptom exhibited by Paul was the slowness and difficult
he experienced when attempting to use his right arm. Gradually the symptom
worsened over the next two years, forcing him to take on a new job. It was then
that he began to realize that he was beginning to suffer from an occasional
shaking of his right arm and leg. The tremor then began to spread throughout
his entire body with the right side being the more severe. Even when he was at
rest the tremors continued to plague him. Paul also began to experience a
decline in his motor abilities. His movement became slower and stiffer, a
phenomena known as bradykinesia. Even simple movements such as rising from a
chair became difficult for him. He began to exhibit the slow, stiff shuffled
gait that is characterized by Parkinson’s disease. His posture became stooped
and his arm swing frequency decreased, as well as the length of his stride. His
arms began to show cogwheel rigidity.
Cogwheel
rigidity is characterized by stiffness in the muscles during flexion and
extension. Tremors are reported as occurring that have a higher frequency level
than those occurring during rest. Tremors present during the cogwheel mechanism
have a frequency of 6 to 9 Hz, while resting tremors typically have a frequency
value of 4-5 Hz (Ghiglione 2005).
Additionally, Paul became to experience micrographia when writing. At
first his writing would appear normal, but over time it will become smaller and
more cramped (Khan, 2011). On top of all that, he began to show
symptoms of finger tapping and rapid alternating movements bilaterally –
meaning both his left and right side did so. He experienced a masklike decrease
in facial expression, another common symptom of Parkinson's. The face becomes
stern looking and the expression rarely changes. His voice became hypophonic,
or softer in tone due to the incoordination of his vocal muscles (hypophonia
2007). Last but not least, he suffered from a retropulsion of two steps when he
was pulled backwards from a standing position. The retropulsion Paul
experienced was caused by the gradual worsening of postural stability and is
associated with the loss of postural reflexes. Retropulsion can lead to the
loss of balance when rising from a chair, which would explain Paul's difficulty
in doing so (Defranco, 2013).
Paul, however, did not experience pronator drift. Patients would
be directed to extend both their arms and have their palms directed upwards. A
rotation of the arm slightly inward and down as well as having a demonstrated a
slight downward drift are symptoms of weakness in the limb. Since Paul did not
demonstrate this however, it is possible to conclude that he had regular
strength in his limbs (Goldberg, 2008).
His coordination was slow, but he did not suffer from ataxia (loss of
muscle control).
After going to a neurologist, Paul was
diagnosed with idiopathic Parkinson’s disease. Typically, Parkinson’s disease
is diagnosed by a careful examination of a patient’s medical history. There is
no specific test developed to test for Parkinson’s disease and since Paul had
no family history of the disease, and no history of use of dopaminergic
antagonist medication, toxin exposure, stroke or encephalitis, other tests were
performed to determine his condition ("Parkinson's
disease health," 2013). Paul’s coordination was tested first, to
test for ataxia. To test Paul’s coordination, finger-to-nose and heel –to-shin
tests were performed. During the finger-to-nose test, patients are asked to
alternately touch their nose and the examiner’s finger, which is held an arm’s
length away from the patient. In the heel-to-shin test, the patients move their
heel up and down the opposite, outstretched leg. At one point in the testing,
the patient would be instructed to keep their outstretched finger close to the
examiner’s, but without coming in contact. Whenever the instructor moves his
finger, the patient is to follow in a parallel fashion. The fingers must be
kept close, but not touching. If the patient undershoots or overshoots the new
position, it is a sign that they have ataxia (Adler & Ahlskog, 2000).
Additionally, Paul’s sense of light touch, pinprick, vibration and joint
position were checked and found to be intact.
Paul
was also subjected to a CT scan and MRI. A CT scan (Computerized tomography)
combines a series of X-ray views taken from different angles and create
cross-sectional images of bones and soft tissues in the body using computerized
technology (Mayo Clinic Staff). The CT
test is used to look for signs of diseases such as Parkinson's in the body ("Parkinson's
disease health," 2013). A MRI (magnetic resonance imaging) scan uses
a combination of magnetism and radio waves, as well as a computer, to produce
images of body structures. MRIs produce detailed images that can be used to
detect small changes in structure. A PET scan is also performed in Parkinson’s
patients, to assess activity and
function of brain regions involved in movement ("Parkinson's disease health,"
2013). Since Wilson’s disease has symptoms similar to Parkinson’s (i.e.
clumsiness, difficulty walking, and involuntary trembling) he was tested for
that as well. In Wilson’s disease, copper is accumulated within the system due
to a genetic mutation in the DNA ("Wilson's
disease: Causes," 2011). However
since he tested negative for the disease, this could not be the cause of his
symptoms.
Parkinson’s
disease has two different types: idiopathic and atypical Parkinson’s. The two
types have similar symptoms. Out of the two, idiopathic Parkinson’s disease is
more common. One of the main ways to determine whether or not a patient has
idiopathic or atypical Parkinson’s disease is to see if the patient responds to
treatments with levodopa or other medications typically used to treat
Parkinson’s. If no response is seen, it is possible that the patient is
suffering from atypical Parkinson’s ("Types of parkinson's,").
Atypical Parkinson’s patients are also more likely to suffer from confusion, and
the disease could lead to the formation of dementia (Jasmine & Zieve,
2011). Patients with atypical Parkinson’s may also lose their balance early on
and have a faster onslaught of symptoms. Symptoms may also be present on both
sides of the body in atypical patients (Giroux, 2011). Paul did not lose his balance early on and did not
demonstrate any symptoms of dementia, leading to the diagnosis of idiopathic
Parkinson’s disease. Additionally, atypical Parkinson’s disease can be drug-induced,
and since Paul has no record of drug use, idiopathic Parkinson’s is the more
likely choice (Giroux, 2011).
There
are two ways to treat Parkinson’s disease. One is through the use of medication
and the other through means of surgery. Paul was treated initially with the
drug levodopa. It is combined with the drug cardibopa to help relieve some of
the symptoms caused by Parkinson’s. After the drug is administered, it converts
to dopamine inside the brain. Cardibopa prevents levodopa from being broken
down in the brain and allows for a smaller dose to be used. This can help ease
certain side effects of the drug such as less nausea and vomiting ("Levodopa and carbidopa,"
2013). Unfortunately, levodopa only proved to be semi-efficient. Paul began to
experience on-off symptoms. The
on-off effect indicates that Paul’s response to levodopa is changing as the
drug progresses. He would suffer sudden fluctuations in movement, from normal
or dyskinesia to parkinsonian slowness and stiffness and then back again (NIH). Paul still had difficulty
walking and carrying out some daily activities, while in the ‘on’ phase of his
medication, and he also had an excessive jerky, involuntary movement of all of
his limbs. The doctors tried both
sustained-release formulations of the medications and a schedule of frequent
small doses, but his on-off symptoms gradually became more severe. Bromocriptine was also used and proved
beneficial. Bromocriptine acts as a dopamine-antagonist and helps stimulate the
nerves that cause movement ("Bromocriptine,"
2013). However, he experienced the same
issues with the Bromocriptine as he did with the Levodopa. Paul signed up for
an experimental treatment of deprenyl (selegiline) plus vitamine E. Selegine can help control Parkinson’s
symptoms by reducing the amount of levodopa required. Selegine helps increase
the amount of dopamine available in the brain ("Selegiline," 2011). Vitamin E can help
Parkinson’s patients by increasing their red blood cell count. It also works as
a blood thinner and can help proper function and growth ("Alternative
treatments for,”). Due to his increasing problems with medicine, Paul’s doctors
suggested a surgical therapy.
Generally, there are only three types of surgery
available for Parkinson’s patients. Paul had a stereotaxic pallidotomy performed
on the left side of his brain
(since his symptoms were worse on the right side of his body). A pallidotomy involves
destroying a part of the brain called the globus pallidus. The over-activity of
the region causes the slowing down symptoms of Parkinson’s. By suppressing the
activity, rigidity could be eliminated, as well as tremor, bradykinesia, and
balance problems. A pallidotomy can also decrease the amount of medication the
patient needs to take. It is by no means a cure, but it can offer relief ("Surgery for parkinson's,”).
Thalamotomy is another surgical option available to Parkinson’s patients;
however it is less popular than pallidotomy. In a Thalamotomy, the thalamus of
the brain is destroyed. Since the thalamus acts as an output area for muscle
movement, blocking it reduces tremors in the body. It, however, is not a
recommended form of surgery ("Surgery for parkinson's,”). Deep brain
stimulation is the final surgical option. During deep brain stimulation, parts
of the brain are made inactive without having to purposely destroy the brain. During
deep brain stimulation, electrodes are
connected by wires to a type of pacemaker device (called an impulse generator,
or IPG) are implanted below the collar bone. Once activated, the device sends
continuous electrical pulses to the target areas in the brain. Electrodes are
either placed in the thalamus or globus pallidus ("Deep brain stimulation,”).
For Paul, the pallidotomy proved to be successful. One day after surgery, Paul his
tremors on the right side and in his left leg were reduced, and he had only a single episode of dyskinesia
(inability to control movements) that lasted about 10 minutes (dyskinesia). He also showed improvements
in his rigidity and had an increase in his walking speed, as
well as larger steps and an
increased arm swing. He still had to take
medication, but after three months he was still doing well. He
continued to enjoy a marked improvement in his symptoms, and
even mentioned that he was “doing
a lot, lot better. I can walk. I can walk straight, and people don’t look at
me like I’m a weirdo anymore.” During
his examination, his
speech was
normal, he had no tremor, only mild bradykinesia, mild dyskinesias of his left leg, and mild slowing of finger
tapping. His gait was
normal and he was able to get up from a chair on his own.
Despite
Paul’s positive results, there are a few downsides to having a pallidotomy
performed. For one, it is not known how long the surgery will last. On the
bright side, however, serious permanent complications are not common. Due to
complications with pallidotomy, it is possible for a patient to suffer from a
stroke caused by bleeding in the brain. Other complications could include an
infection or seizures ("Pallidotomy
(posteroventral pallidotomy)," 2010).
It
is not known how long the surgery lasted for Paul, but his results seemed
promising after the surgery. On a personal level, I do not believe surgery is always
the best option, but in Paul’s case (and the case of many other patients with advanced
Parkinson’s disease) it was. I do, however, prefer surgery over taking
medication (in extreme causes such as Parkinson’s disease). While surgery will
not cure Parkinson’s or get rid of the need for medication, it can reduce the
amount he has to take, which can reduce any nasty side-effects he could receive
from long-term use. One of the side effects of prolonged use of levodopa is
dyskinesia ("Carbidopa/levodopa,").
In the end he is still taking medication, but this way he can enjoy the better
side effects of taking the drugs, without experiencing the negative side
effects.
Citations
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