Understanding Parkinson’s Disease

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Parkinson’s disease is one of the most common degenerative brain disorders today. It was previously called as “shaky palsy” when Dr. James Parkinson identified the disease in 1817. It was called as “shaky palsy” because of the characteristic sign of shakiness among people suffering from the said disease.

Parkinson’s disease occurs when the brain cells progressively injures and degenerates. When this happens, our brain cells die and lose function. The exact cause of this disease in unknown however, many scientists point out to its connection to genetics and environmental factors.

Pathophysiology of the Disease

Our muscular movements are greatly affected by neurotransmitters—these neurotransmitters send signals from the brain to the area of the body which needs response. One important neurotransmitter affected in Parkinson’s Disease is Dopamine. Dopamine is produced in many areas of the brain but one important area where bulk of Dopamines is produced is the Substantia Nigra.

This crescent shaped area called the substantia nigra is the one damaged in the event that Parkinson’s Disease develops. With a significant damage to this area, Parkinson’s Disease starts to manifest. In fact, Parkinson’s Disease’s signs and symptoms will become obvious when 80% of the cells in the substantia nigra degenerates—meaning, remedying PD may be hard since it may present as a full blown condition.

Signs and Symptoms of Parkinson’s Disease

Difficulty moving and loss of movement coordination is a common symptom Parkinson’s Disease. This is due to the loss of Dopamine which is responsible for many of our gross and fine motor skills. This is most evident when a person walks in a shuffling gait.

Shakiness and tremors are also common symptoms of PD. This is the most common early sign of PD and may occur initially in the fingers, hands, arms and may progress to the face, jaw and legs.

Muscle stiffness is also one of the cardinal signs of PD which may leave a patient with PD unable to move his or her arms and legs altogether.

Diagnosis of Parkinson’s Disease

Most cases of PD are diagnosed with the presence of the signs and symptoms that will point out to the specific disease. A CT Scan may also be helpful in identifying diseased areas of the brain.

Treatment of Parkinson’s Disease

PD may be treated mainly with the use of drugs. Drugs used for this disease are those which increase the levels of Dopamine or mimic the effects and actions of Dopamine. Examples of such drugs are Levodopa, Bromocriptine, etc.

Specific treatments for patients with PD may include therapy like speech therapy, occupational therapy and physiotherapy. These forms of therapies will help the patient with PD maximize his or her functions and helping him or her attain a good sense of independence from caregivers.

Needless to say, a patient with Parkinson’s Disease needs as much supervision as possible because of the physical injuries that may occur. Many people with this disease can go on with their usual routine, while there are also some whose daily activities have been significantly affected. Getting prompt treatment for this disease is important in promoting longevity and quality of life in a patient with Parkinson’s Disease.

 

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  1. I thought you might be interested in seeing the following essay which I wrote and presented last month at the April 2011 “Men’s Brotherhood Breakfast” in Suches, GA,

    “April is Parkinson’s Disease Awareness Month “Version 2.0”
    (I wrote “Version I” in April of 2010.)

    To those of you who were here and remember the “Version I” presentation I made on this topic last year, I ask your indulgence for a few moments while I recap some of the fundamentals for the benefit any newcomers who might not be familiar with it.

    For a long time, the Parkinson’s community had recognized April as Parkinson’s Awareness Month, but it was only in March of 2010 that we received the US Senate’s imprimatur. And, because I have had the disease since 2004, and am an advocate for awareness, I want to do something to call attention to this chronic, progressive, idiopathic neurodegenerative disease for which there is no cure. That’s doctor-speak for “a disease that kills more brain cells every day, but they do not know what causes it, or how to cure it, reverse it, stop it, or slow its inexorable progression.” And we are not a small group: I am one of perhaps more than six million world-wide, and as many as two million Americans, who are living with PD (Parkinson’s Disease) today. To put that in perspective, more Americans live with PD than the combined number of people diagnosed with Multiple sclerosis, Muscular dystrophy, and ALS ,or Lou Gehrig’s disease; and, the number of Americans with Parkinson’s disease increases annually by about 50 ~ 60K.

    If you have stuck with me this far, I hope it is because you are interested in “raising the awareness” about this disease. And, I hope you find my comments useful.

    Those of you who are familiar with the presentation I made here last year may recall my mentioning that persons with Parkinson’s Disease (“PwPs” for short, also known as “Parkinsonians”), are like snowflakes – no two are exactly alike. The rate of progression of the disease varies by patient, and it is not possible to accurately predict the likely course in an individual case: in the best case scenario, a person may enjoy many years of productive living (and I thank God that so far, I’m on that track); in the worst case scenario, a person may quickly suffer serious problems such as failing memory, unintelligible speech, disabling bradykinesia, cachexia, dementia, invalidism, and death. Such cases are not common, but they do happen.

    And unfortunately, although medication, and sometimes surgery, can help mask the symptoms, nothing we have can alter the progressive nature of the disease. And by progressive I mean, the original symptoms get worse over time, and previously unobserved symptoms begin to appear.

    PD is basically (but not only) a movement or motion disorder involving progressive degeneration of nerve cells in the area of the brain controlling muscle movement, which results in the appearance of certain motor symptoms. The four cardinal motor deficiencies associated with PD are:

    tremor (of the hands, arms, legs, jaw or face);
    bradykinesia (slowness of movements and reflexes);
    rigidity (stiffness of the limbs and trunk);
    postural instability (impaired balance and coordination, instability when standing, difficulty walking, and stooped posture.)

    Less typical motor deficiencies may include: small, cramped handwriting; shuffling walk; soft, muffled or slurred speech; difficulty swallowing; fatigue; impaired fine motor or gross motor dexterity and coordination; reduced arm wing; sexual dysfunction; rocking, squirming, restlessness; muscle cramping; drooling; and a fixed, inexpressive face.

    As I mentioned earlier PD is not only a movement disorder, a fact which is not widely known by the general public. There are many PD non-motor deficiencies and the International PD Non-Motor Group, a nonprofit academic organization headquartered in the UK, has developed and validated a patient questionnaire listing 30 non-motor symptoms , The questionnaire is useful because for many PwPs, the non-motor deficiencies are more troubling than the motor symptoms,

    Examples of non-motor deficiencies include:
    Depression, loss of energy, sleep disturbances, constipation, pain, fear or anxiety, confusion, dementia (which affects one-third of PwPs), memory difficulties, slowed thinking, compulsive behavior (usually gambling or shopping), and cognitive Impairment – At any given time, nearly all PwPs have some form of cognitive impairment, which involves memory disturbances, attention difficulties, slowness in information processing, language dysfunction, and visual-spatial disturbances.

    The total list of motor and non-motor symptoms is quite long, e.g., the Movement Disorder Society-sponsored revision of the Unified Parkinson’s Disease Rating Scale (MDS-UPDRS), which is used extensively in clinical investigations, lists more than 50 motor and non-motor symptoms. This essay includes only some of the most common symptoms.

    The PD community has seen some significant medical advances in the last year and I would like share with you, what I consider to be some of the more important or more interesting changes and advances.

    Current evidence suggests that:

    … PD strikes men more than twice as frequently as it does women.

    … Whites and Hispanics are twice as likely to suffer PD as African Americans and Asians; however, African Americans tend to have greater disability than whites.

    … lower income and lower educational level are associated with increased incidences of PD, as well as higher severity and level of disability.

    … PD is more common in the Midwest and the Northeast where chemicals used in metal processing and agriculture present potentially serious environmental risk factors for PD. .

    … about 95% of cases are still “of unknown cause,” and research continues in many areas, with much focus on genetics, environment, and lifestyle.

    During the past year, research focusing on the relationship of genetic factors has received much attention. One international study identified eleven different low risk genes involved in noninherited forms of PD. The top 20% of people carrying several of these genes are 2.5 times more likely to develop PD than those with the fewest low risk gene variants.

    In the area of stem cell research, there are actually two basic kinds of stem cells: adult stem cells (which are present in bone marrow), and embryonic stem cells. The latter are the target of the imbroglio in the USA, which seems not to be an issue in places like China (which has achieved the fastest development in stem cell research of any country during the past 10 years), India, Mexico, Germany and Thailand, and even though stem cell transplants are still considered experimental in the USA, some other countries are already performing stem cell therapies, promoted by international advertising campaigns to entice people with serious diseases, e.g., ALS (Lou Gehrig’s disease), spinal cord injuries, Alzheimer’s, cerebral palsy, MS, PD and many cancers. Some of these therapies are legitimate, and some are not. If you, or someone you know, is planning a trip out of the country for stem cell therapy please first consult with The International Society for Stem Cell Research, where leading stem cell researchers can review a specific clinic and evaluate its claims. You might also want to check on the status of the development of a noninvasive cell therapy delivery via the nose, based on successful intranasal delivery of stem cells to the brains of rats with PD, which resulted in substantial improvement in motor function. An international team of researchers conducted the study, which was published in the Rejuvenation Research Journal .

    Studies of environmental factors confirmed that people who used either of two pesticides, rotenone and paraquat (Gramoxone), developed PD 2.5 times more often than people who never used the pesticides; people exposed to high levels of manganese pollution had a 78 percent greater risk of developing PD than those living in areas free of such pollution; and, people living in areas with high levels of copper pollution had an 11 percent greater risk of developing PD.

    In my wrap up, I’d like to briefly summarize a few ongoing studies which indicate to me that we are getting closer to the day when there will be a neuroprotective therapy for humans. Where it will come from, no one knows for sure. Some advocates, and organizations, expect the answer will come from gene research; others feel the same about environmental factors research, or chemical therapies; others are focusing on lifestyle factors; and some are studying proteins and vitamins. For the most part, they are investing their money and efforts in the areas they feel are the most promising and some are invested across the board. Here are a few examples:

    SR-3306.
    (subtitle) Not a cure, but a step in that direction.
    Scientists have developed a compound – named SR-3306 –that can treat the brain cells in mice and rat PD models and slow or stop the progression of the disease. This is the first compound known to show significant efficacy in blocking brain cell destruction in PD, unlike virtually all other current therapies which only treat the symptoms of PD, and not the actual neurodegeneration. This is merely the first step in the lengthy FDA process required before a new drug can be brought to market. So we must be patient.

    Phenylbutyrate and DJ-1
    (Subtitle) A drug – gene cooperative effort.
    Perhaps the second most exciting announcement came just recently (March 9, 2011) when researchers at the University of Colorado School of Medicine announced the discovery of a drug that stops the progression of PD in mice, and is now being tested in humans. The drug is called phenylbutyrate and it activates a gene (the DJ-1 gene) in the brain which keeps dopamine neurons from dying.

    Anthocyanins.
    (subtitle) We always knew berries were good for us.
    The results of a Harvard Medical School study suggests that people who consume foods rich in flavonoids and a flavonoid subclass called anthocyanins, which are found primarily in blue berries and strawberries, may be protecting themselves against developing PD. The study concluded that people who consumed the most anthocyanins were significantly less likely to develop PD than those who consumed the least.

    BEE VENOM.
    (subtitle) Until now, I didn’t think there was anything good about a bee sting.
    A few years ago researchers discovered that bee venom and/or its constituent, apamine, are able to slow or halt the disease process in animal models of PD. Recently, researchers in Korea working with mice have corroborated the findings.
    DOPAL .
    (subtitle) “We have met the enemy and he is us!” (With apologies to Pogo.)
    PD motor symptoms begin to emerge only after about 80 percent of the dopamine producing cells in the brain prematurely die or become damaged. Earlier this year, researchers reported finding evidence that the natural brain toxin DOPAL is the killer. DOPAL attacks healthy cells and leads to the crumpling of a protein called alpha-synuclein, which increases DOPAL, leading to further reductions of dopamine producing cells, leading to less dopamine, resulting in more PD symptoms, and it is this viscous cycle that accounts for the progressive nature of PD. As you can imagine, this information is quite valuable in the pursuit of new therapies.

    WS-CoQ10.
    (subtitle) Not alone, but with vitamin E.
    Early this year researchers began to conduct a clinical study of the neuroprotective value of a combination drug composed of CoQ10 plus vitamin E called WS-CoQ10, which has done well in providing protection for dopamine nerve cells in pre-clinical models of PD.

    And finally, some of the best news is for wine & cheese lovers:
    Azilect® + red wine + aged cheese = OK
    The original prescribing information for Azilect® (rasagiline tablets) – which is in use today as PD therapy, and is also currently under study as a possible neuroprotective agent – included dietary restrictions against consumption of foods high in tyramine, a combination which, it was thought, had the potential for hypertensive crisis – which in some cases could be fatal. Included on the restricted list were red wine and aged cheeses, two of my favorites. Happily, following further study, the FDA lifted the restrictions, freeing me (and all other cheese and wine aficionados), to follow my father’s favorite dictum: una pranzo senza vino e come una giornata senza sole. (That’s Italian for “A meal without wine is like a day without sunshine.”)

    And on that happy note, I’ll conclude my essay, and my presentation. Thank you for your attention

    = = E N D= =

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