Parkinson's Disease is a sporadic disorder, with a probable genetic predisposition in ~16% of  families. The average age of onset of symptoms is 63. The range of age of onset is quite wide, with "young onset" Parkinsons occurring not infrequently. 8% of patients have the onset of their symptoms before age 40.

There are no blood tests or X-rays that will confirm the diagnosis. The diagnosis is made on finding 2 of the 3 cardinal features of the disorder on neurologic exam and ruling out other possible causes includung several conditions that can mimic Parkinson's Disease but often have additional features (Parkinsn's "Plus").

Cardinal Features (T.R.A.P.)

1) Tremor:

This is present in 80% of cases. It is usually manifest by a tremor at rest (at a time when the body part is relaxed). It may affect the hands, feet, chin, and tongue. It doesn't affect the head.  Classically when it affects the hand it creates a "pill rolling" tremor. The thumb slides back  and forth on the index finger.  30% of patients will also have a component of this tremor with action (movement induced tremor). This tremor like all tremors will be worse after physical  exertion, or with good or bad emotional excitement.

2) Rigidity (stiffness):

 Rigidity or stiffness in muscles leads to a characteristic loss of arm swing with walking, loss of facial expression, fatigue, and a variety of muscle pain syndromes (e.g. frozen shoulder). This is  identified on examination by the examiner passively manipulating the limb while the patient is relaxed. Getting the patient to perform movements with the opposite side of the body will  accentuate the abnormality in the tone of the limb. There is  a classically described "cog wheel" type of rigidity in Parkinson's Disease. Without regular exercise, physiotherapy, and/or medication this rigidity can lead to progressively increased problems with immobility and muscular pain.

3) Akinesia (slow movement):

A general slowing down of movement will occur. This together with the rigidity causes the  many other symptoms, and signs of Parkinson's. The walking slows, the feet start to shuffle, the voice softens, and losses its expression. Fine coordinated tasks like buttoning and cutting  food become difficult. Writing becomes messy, and gets smaller, and increasingly illegible.

Other symptoms:

A stooped posture and a blank facial expression occurs as the result of immobility and rigidity.

Drooling is often an early symptom with the pillows being wet at night. This isn't due to increased salivation, but relates to decreased frequency of swallowing.

Speech problems result from incoordination or reduced movements of the muscles involved in breathing, voice, pronunciation and prosody (rhythm, intonation and speaking rate). The first  change is usually a soft or fading voice.  Words may become slurred or unclear with the final sounds in words being omitted. Speech may become faster. Their may be difficulty beginning  conversations or sentences causing hesitations or uncontrolled repetitions of words or phrases. Drooling may cause muffled speech. 

Seborrhea or excessive scalp, eyebrow or eyelash oiliness, reddness, or scaliness may occur.

Constipation is often a major problem. It results from a combination of  immobility, slowing of  digestion, and antiparkinson medications.

Sexual dysfunction is a frequent problem due to the autonomic nervous system involvement.   Impotence as well as hypersexuality may be a side effect of antiparkinson medications.

Easy fatigability is often the result of the effort and constant attention required to move. This is complicated by sleep disturbances including sleep initiation problems and early morning wakening that may result from depression. Insomnia may occur from antiparkinson medication, as can vivid dreams or night mares.  Parkinson patients are more likely to act out their dreams.

 Postural Instability (loss of balance) occurs later in this condition.

Late in this condition problems with low blood pressure causing weakness or dizziness can occur.

Freezing or sudden loss of movement, often occuring in doorways, or when attempting a  movement that requires more than one action (such as walking to a door and opening it) may trigger this. Cues such as seeing something to step over, hearing a beat to move with, or having  someone touch the individual  may help the person to start moving

Difficulty swallowing affects approximately 50% of advanced parkinsonians. It is caused by  slow moving, poorly coordinated throat muscles.

Sweating , or an impaired sweating response may occur. Antiparkinson medications may aggravate this.

Bladder problems occur in advanced parkinson's, and results from varying effects of the illness on sphincter muscle control.   Antiparkinson medication may cause retention of urine and constipation.

Mental changes including depression which occurs in approximately 30% of Parkinson patients may be aggravated by self-imposed social isolation. Fatigue may contribute to feelings of discouragement. Anxiety may also be evident.

Memory loss will occur in up to 30-50% of patients. This occurs late in the illness.

Progression:

The disorder progresses differently from one individual to the next. With treatment almost normal abilities can be maintained for 5-10 years. Unfortunately there is no curative treatment  and the treatment options only help control the symptoms.

As the illness progresses the patients require more medications at a time when they are less  able to tolerate their side effects. This can lead to fluctuating degrees of control of the symptoms. Variable degrees of disability start to break through the previous ability to maintain  consistent symptom control throughout the day.

Most of the ongoing research is investigating ways of pushing this problem further into the future, and to lengthen the time the symptoms can be adequately controlled.

Some problems unfortunately have no treatment (e.g. memory loss).

Cause:

The cause is unknown. It is known that Parkinson patients have a marked reduction in the number of nerve cells in the Substantia Nigra (black pigmented nerve cell in the brain stem). Some families are known to be have an inherited defect that is assumed to result in a failure of  survival of these nerve cells. These cells are known to die off earlier than expected in Parkinson's Disease. Scientists, and Doctors still don't know why. This results in the chemical that these cells produce,  dopamine, becoming deficient. Without dopamine the part of the brain that normally slows down excess movement becomes over active, resulting in the slowness and stiffness seen in this disorder.

Treatment:                                            Work Toward a General Healthy Lifestyle:

There are currently several options to treat Parkinson's disease.

The first step is to make sure the patient becomes well informed about the disorder.

A healthy lifestyle with good eating, sleeping, and exercise habits is strongly encouraged.

Early assessment by a physiotherapist to initiate an appropriate regullar exercise program is recommended.

The motor vehicle branch needs to be informed, but early on the illness should not interfere with safely driving a motor vehicle if the expected treatment response occurs.

Medication Treatment Options:

The 2 main options with treatment include replacing dopamine directly with L-dopa (Dopamine given directly is poorly absorbed. L-dopa is a precursor which is well absorbed), or using a chemical mimic of dopamine (a dopamine "agonist").

 L-dopa comes in a mixture together with carbi-dopa or benzerazide. These other chemicals reduce the nausea that would occur if L-dopa was given alone. These medications come under the trade names of Sinemet, Sinemet CR, and Prolopa. Common side effects include nausea, dizziness, and confusion. hallucinations, and abnormal involuntary movements.

There are 3 dopamine agonists available in Canada, each varying in cost, duration of action, and side effects. In general the side effects are similar to l-dopa. The dopamine agonists available include: Bromocriptine, Ropinirole, and Pramipexole.

Other medication options include some older medications including Amantadine (symmetryl), and the anti-cholinergics (e.g. trihexyphenidyl, benztropine, ethoproprazine).

Amantadine will help symptoms 60% of the time. It's duration of benefit varies. Recently it has been helpful in treating the dyskinesias (abnormal involuntary movements) that may occur  with the above medications.

The anti-cholinergics can be helpful for refractory tremor, and tend to dry up saliva, so they  can reduced drooling. Overall  these medications are less well tolerated particularly in older patients due to their tendency to reduce memory.

Selegiline is a medication that blocks the breakdown of  dopamine, and may prolong the benefit patients receive from l-dopa medications. There had been some suggestion that  selegiline might slow the progression of Parkinson's disease in the late 1980's, but most neurologists consider this as unproven as a result of several trials that have shown inconsistent results.

Avoid Drugs that are Contraindicated in Parkinson's:

The term "contraindicated" essentially means that the drug in question should not be given to a patient. In the parkinsonian, the basic problem is the chemical nature of the disease itself. Many  drugs alter the brain's dopamine system and may not be recognized as having the potential to markedly alter the symptoms of Parkinson's simply because the drugs are often used for the  treatment of non-neurological conditions. Every patient with Parkinson's should have a list of these agents available for their physician's reference.

Information in Table above taken from The Transmitter Vol. 7, No. 4, Oct 1990.

Medication Information Link:

 www.rxmed.com/b.main/ b2.pharmaceutical/b2.prescribe.html or

  www.nlm.nih.gov/medlineplus/druginformation.html

Surgical Options:

In addition to pills surgical treatments are now more frequently used to help control symptoms that become refractory to medications.

1)  Steriotactic thalmotomy is a procedure that surgically destroys the very small area deep within the brain that causes the tremor (the thalamus).   This, if successful, will stop the tremor on the opposite side of the body.   There are, as with any surgery, possible side effects that need to be discussed and considered.

2) Another option is to implant a deep brain stimulator  (a small electrode usually implanted into the Sub-thalamic nucleus, that is connected to a programmable transmitter under the chest wall, like a pacemaker).  By electrically interfering with the natural firing pattern of the nerve cells in this area the tremor is electrically suppressed and in addition the rigidity and slownwss of movement also improves.

On average with this type of surgery there is an average 50% reduction in medication and a smoothing out of the fluctuations in response to medication. There has now been > 12 year follow-up and many examples of maintained improvement over the long term.  There are, as with any surgery, possible side effects that need to be discussed and considered.

Surgical procedures directed to controlling other symptoms by operating on, transplanting, or stimulating other areas of the brain are continuing to produce exciting results.

Follow this link to more information on Deep Brain Stimulation:

www.medtronic.com/neuro/parkinsons/activa_qa.html