The Shaking Palsy

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What is the Shaking Palsy?

Parkinson's Disease, first described in 1817 by British physician James Parkinson, who called it "the shaking palsy", is a slowly progressive, degenerative brain disorder, that leads to slow and reduced movement, rigidity of the muscles, tremors at rest and gait (walking and postural) instability.

How prevalent is Parkinson's Disease?

It is the fourth most common brain deterioration disorder in the elderly. About 1% of those 65 and older, and about 0.4% of those older than 40 are affected. However, Parkinson's Disease could also begin in childhood or adolescence. Ten percent occur under age 40. Generally, the onset of symptoms occur after the age of 40 and the incidence goes up among older people.

Who are some well-known personalities afflicted?

Pope John Paul II, U.S. Attorney General Janet Reno, Rev. Billy Graham, actress Kathryn Hepburn, sitcom actor Michael J. Fox, former heavyweight boxing champion Muhammad Ali, country singer Johnny Cash.

What causes Parkinson's Disease?

The cause of the Primary form of Parkinson's is not known, hence it is called idiopathic. Secondary Parkinsonism could be due to other forms of degenerative disease of unknown cause, or due to drugs or external toxins. The most common is ingestion of antipsychotic drugs or reserpine, which blocks dopamine receptors in the brain causing Parkinsonism. The less common causes are carbon monoxide or manganese poisoning, encephalitis, tumors, bleeding in the brain, and use of illegal IV drug called N-MPTP, a drug synthesized from Meperidine.

Exactly what happens in the brain?

The brain is the most sophisticated, elaborate and complex communications system there is in the world. Not even the fastest computer available today can match, much less equal, the human brain. Parts of the brain called substantia nigra, locus caeruleus and brain stem have dopaminergic nerurons (cells that produce dopamine, a neurotransmitter that helps direct muscle activity). In Parkinson's, there is loss of these neurons that produce dopamine and the resultant depletion of dopamine characterizes Parkinson's Disease. Without enough neurotransmitters, it is obvious that vital normal "connections" are impaired, which explains why these patients act and behave a certain way, presenting typical signs and symptoms of the disease.

How does the disease start?

In 50 to 80 of Parkinson patients, the condition starts insidiously with the so-called "pill-rolling" tremor of one hand. This is very typical. The uncontrollable coarse tremor looks like the fingers are rolling a pill or a marble continuously. This is severe at rest and decreases during movement, and is not present when the patient is asleep. Emotional stress, tension or fatigue aggravate it. The hands, arms and legs are commonly affected, in that order. In some patients, tremor is absent, and rigidity of the muscles is present. The face becomes mask-like, expressionless, with diminished blinking and with mouth open.

How is walking affected?

The patient initially has difficulty starting ambulation. The gait becomes shuffling with short steps, with the hands flexed to the waist and do not swing with walking. The steps may gradually quicken beyond the patient's control, and the patient may find himself running to prevent falling because of instability of his gait. Sometimes, he could only stop when he hits an object. His center of gravity is displaced forward (or backward) due to loss of postural reflexes.

What are the other symptoms?

The speech among Parkinson patients becomes hypophonic (monotonous) and stuttering. The legs have led-pipe rigidity when the physician examines and moves them. The sensation and muscle strength are usually normal. Depression is common among these patients, and 50% of them develops dementia, like Alzheimer's. Constipation, urinary hesitancy, seborrhea and orthostatic hypotension (low blood pressure when they stand up from a sitting or lying position) are common among these patients because of autonomic nervous system dysfunction.

Is there a way to prevent Parkinson's Disease?

Since Primary Parkinson's Disease is idiopathic (cause unknown), there is no way to predict or prevent it as yet. Scientists are now searching for a biomarker, a biochemical abnormality these patients might have in common, so screening diagnostic test might be developed to detect Parkinson's even before symptoms develop. PET (Positron Emission Tomography) brain scanning may eventually lead to advances in our understanding about this baffling disease. Secondary Parkinsonism to a great extent is preventable by avoiding those conditions and drugs mentioned earlier that cause it. One general health rule that applies, and must be observed especially in this particular situation, is: Stop abusing our mind and body by living a healthy lifestyle.

Do hand tremors pre-sage Parkinson's Disease?

The usual hand and finger tremors we see among older persons, and some even among those who are younger than 40, do not necessarily mean those individuals will develop Parkinson's Disease later in life. These so-called "normal" tremors or shakes do not indicate the presence of a disease. However, if they are so severe as to affect the daily normal use of the hands, a neurological evaluation might be in order.

What is the treatment for Parkinson's Disease?

Drug therapy to replace the mission neurotransmitter dopamine, to stimulate dopamine receptors, to inhibit the major enzymes that break down the remaining dopamine in the brain, and drugs to control the involuntary movements, rigidity and tremors is the standard treatment. Rehabilitation and Physical therapy are most helpful. Since the inactivity due to Parkinson and the constipating side-effects of the medications, high fiber diet and stool softeners are part of the standard regimen.

How about surgery?

Surgery was once very common in treating Parkinson patients, but when Levodopa was discovered, this drug became so effective surgery was confined to only few cases. Most patients respond to anti-Parkinson medications, but these drugs may lose their efficacy over time. Stereotactic ablation (surgical cutting), cryothalamotomy (insertion of supercooled metal probe into the thalamus (relay station deep in the brain) to destroy the part of the brain that produce tremors, results in significant improvement. Some patients enjoying sustained improvement for up to 4 years following surgery. Pallidotomy, another procedure using the same principle as above, is creating renewed interest among neurosurgeons. High frequency electrical stimulation of the globus pallidus appears to be promising, and so with deep brain stimulation. These procedures are under further scientific evaluation. Fetal dopamine neuron transplantation is another exciting option under study.

©2003Raoul R. Diez, M.A.O.D.