PARKINSON'S DISEASE
WHAT IS PARKINSON'S DISEASE? (PD)
Parkinson's disease is named after Dr.
James Parkinson, a British Physician, who wrote a famous description of the
disease called "Essay on the Shaking Palsy" in 1847.
PD is a progressive degenerative condition
of the brain which preferentially affects a specific group of nerves in an
area of the brain called the "substantial nigra pars compacta". This area of
the brain is responsible for producing a brain chemical called dopamine
which is critical in control of movement. When this brain chemical is
deficient, the signs of PD are present including tremor, rigidity of the
limbs and body, slowness or lack of movement (bradykinesia) and walking
difficulties characterized by a flexed posture and short shuffling steps
(petit pas gait). People with this disease may develop episodes of sudden
inability to continue or initiate a movement, as well as reduction in the
usual animated facial movements, blinking frequency and normal arm swing
while walking. Their writing may become small and difficult to read (micrographia).
The voice may become soft and quiet (hypophonia). Symptoms often begin on
one side of the body and later spread.
PD is usually a progressive disorder and
gradually worsens over time. It is frequently associated with depression
which can occur at any time during the course of the disease. Later in the
disease, up to 30% of patients may develop dementia. Some patients also
complain of difficulty with peeing, waxy build upon the skin (seborrhea),
and a deep achy sensation in the limbs.
WHAT CAUSES PARKINSON'S
DISEASE?
The exact cause of PD is not known. A
somewhat similar disease pattern can be produced due to the toxic effects of
certain drugs on the substantia nigra raising this as a possible mechanism.
Infectious causes and a possible genetic pre-disposition have also been
proposed as contributing causes.
PD occurs most commonly in the elderly
(>60 yr.) but can be seen in younger adults and very rarely in juveniles.
15% of cases tend to run in families. Recent research suggests that this may
be due to an abnormal gene on chromosome 6.
CAN OTHER PROBLEMS LOOK
LIKE PD?
Yes. PD is one of a group of degenerative
conditions that may present in a similar fashion including progressive
supranuclear palsy (PSP), striatonigral degeneration (SND), and
multi-systems atrophy. In addition, several medications, especially the anti
psychotic type can produce a PD-like picture.
HOW IS PD DIAGNOSED?
The diagnosis of PD is usually made based
on the patient's history and physical examination. Additional testing to
rule out other causes of Parkinson's syndromes can be helpful including
CT scanning of the
head and positron emission tomography (PET) in atypical cases.
WHAT CAN I DO ABOUT IT?
Although there is no cure for PD at the
moment, there are several therapies available that are quite effective in
controlling the symptoms and that improve function for quite an extended
period of time. Exercise and mental activity are important in maintaining
function and flexibility and in reducing side effects such as
depression.
MEDICAL(DRUG)THERAPY
Most patients begin treatment when their
symptoms begin to interfere with their work or daily life activities. A
variety of medications are useful. These are primarily aimed at
replacing or mimicking the activity of dopamine in the brain. The initial
choice of medication depends on the patient's overall medical condition and
age with the general goal to use the minimum dose of anti-parkinsonian
medication necessary to control symptoms.
The main groups of medications for
treating PD including Carbi-dopa/Levo-Dopa combination drugs (e.g. Sinemet),
dopamine agonists (e.g. bromocriptine, pergolide, ropinirole, pramipexole),
catachol-O-methyltransferase (COMPT) inhibitors (e.g. Entacapone),
Amantadine and anticholinergics (e.g. Trihexyphenidyl Hydrochloride,
Benztropine).
The dopamine agonists are often the
initial choice for therapy since there is some evidence that using these
agents alone may delay or reduce later complications of PD. Levo-dopa/Carbi-dopa
combinations are often more powerful drugs for the treatment of slowness and
stiffness and may be the first choice if the patient has medical problems or
impaired cognition. Anticholinergic medications are primarily effective for
the tremor, but tend to have problematic side effects including blurred
vision, dry mouth, confusion and memory difficulties that limit their
usefulness, especially in older patients.
You should discuss with your physician
what drugs would be most appropriate and what the overall strategy for
treatment is.
SURGICAL THERAPY
Surgical therapy is usually considered in
patients whose symptoms are no longer controlled with medical therapy. The
type of surgical therapy depends upon the primary symptom. Thalamotomy
(placement of lesions in the thalamus) has been used in the treatment of
tremor, but has mostly given way to deep brain stimulation due to the high
incidence of complications such as speech problems.
Pallidotomy,
placing lesions in the internal segment of the globus pallidus (GPi) can
improve tremor, rigidity and slowness of movement. When done on both sides
of the brain however, it can lead to complications of softness of speech (hypophonia)
which make it less useful when the disease is prominent on both sides.
Deep brain stimulation
involves placing an electrode within the GPi or subthalamic nucleus (STN)
and connecting to a stimulator under the skin. This can be effective for
both tremor and the stiffness and slowness of PD and can be performed on
both sides of the brain.
Brain cell transplantation remains an
experimental therapy of uncertain usefulness.
Surgical treatment of PD requires evaluation at a centre
and with a surgeon who is familiar with the treatment. In British Columbia,
this is only taking place at
Vancouver
General Hospital at the moment.
Dr. R. O'Brien
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