Parkinson's Disease Case Study

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

Adler, C., & Ahlskog, J. (2000). Parkinson's disease and movement disorders. (p. 14). Mayo Foundation for Medical Education and Research.

Alternative treatments for parkinson's disease. (n.d.). Retrieved from http://www.webmd.com/parkinsons-disease/guide/alternative-treatments-parkinsons

Bromocriptine. (2013, November 26). Retrieved from http://www.nlm.nih.gov/medlineplus/druginfo/meds/a682079.html

Carbidopa/levodopa. (n.d.). Retrieved from http://www.parkinson.org/Parkinson-s-Disease/Treatment/Medications-for-Motor-Symptoms-of-PD/Carbidopa-levodopa

dyskinesia. (n.d.) The American Heritage® Dictionary of the English Language, Fourth Edition. (2003). Retrieved December 6 2013 from http://www.thefreedictionary.com/dyskinesia

Deep brain stimulation for parkinson's disease. (n.d.). Retrieved from http://www.webmd.com/parkinsons-disease/guide/deep-brain-stimulation

Defranco, M. (2013, September 5). Physical therapy tips to manage retropulsion in parkinson’s disease. Retrieved from http://mdc.mbi.ufl.edu/treatment/physical-therapy-tips-to-manage-retropulsion

Ghiglione P, Mutani R, Chiò A. Cogwheel Rigidity. Arch Neurol. 2005;62(5):828-830. doi:10.1001/archneur.62.5.828.

Giroux, M. (2011, March 24). Atypical parkinsonism. Retrieved from https://www.nwpf.org/wellness/PhysicalHealth/ParkinsonsHealth/PDTipsDetail.aspx?id=220&headerbar=1

Goldberg, C. (2008, August 16). A practical guide to clinical medicine. Retrieved from http://meded.ucsd.edu/clinicalmed/neuro2.htm

Heyn, S. (n.d.). Retrieved fromhttp://www.medicinenet.com/parkinsons_disease/article.htm

"hypophonia."Dorland's Medical Dictionary for Health Consumers. 2007. Saunders, an imprint of Elsevier, Inc 3 Dec. 2013 http://medical-dictionary.thefreedictionary.com/hypophonia

Jasmine, L., & Zieve, D. (2011, September 26). Secondary parkinsonism. Retrieved from http://www.nytimes.com/health/guides/disease/secondary-parkinsonism/

Khan, U. (2011, April). Parkinson disease: Micrographia. Retrieved from http://lifecenter.ric.org/index.php?tray=content&tid=top427&cid=3780

Levodopa and carbidopa. (2013, November 26). Retrieved from http://www.nlm.nih.gov/medlineplus/druginfo/meds/a601068.html

Mayo Clinic Staff. (n.d.). Retrieved from http://www.mayoclinic.com/health/ct-scan/MY00309

NIH. (n.d.). Retrieved from http://nihseniorhealth.gov/parkinsonsdisease/medications/01.html

Pallidotomy (posteroventral pallidotomy) for parkinson's disease. (2010, December 3). Retrieved from http://www.webmd.com/parkinsons-disease/pallidotomy-posteroventral-pallidotomy-for-parkinsons-disease

Parkinson's disease health center. (2013). Retrieved from http://www.webmd.com/parkinsons-disease/guide/parkinsons-diagnosis-tests

Parkinson's disease: Hope through research. (2013, November 21). Retrieved from http://www.ninds.nih.gov/disorders/parkinsons_disease/detail_parkinsons_disease.htm

Selegiline. (2011, March 06). Retrieved from http://www.nlm.nih.gov/medlineplus/druginfo/meds/a697046.html

Surgery for parkinson's disease. (n.d.). Retrieved from http://www.webmd.com/parkinsons-disease/guide/parkinsons-surgical-treatments

Types of parkinson's and parkisonism. (n.d.). Retrieved from http://www.parkinsons.org.uk/content/types-parkinsons-and-parkinsonism

Wilson's disease: Causes. (2011, September 23). Retrieved from http://www.mayoclinic.com/health/wilsons-disease/DS00411/DSECTION=causes