Multiple System Atrophy
By Tim Foley
Multiple System Atrophy, also
known as Multi System Atrophy is a severely debilitating Parkinsonism. I
have spent some time now trying to increase awareness of this disease
amongst the PD’ers. Many times people will confuse MSA with Multiple
Sclerosis and Muscular Dystrophy. Someone once told me that at least I
could find support through Jerry Lewis’ Organization. This was from a
person with PD and at that point, a bell went off in my head.
We are far from finding any cure
for MSA as it involves multiple areas of the brain. MSA is also a rare
disease so it is quite uncommon to know someone with this disease.
MSA is a very humbling but
unforgiving disease. Symptoms can be slowed down early in the game with
medications but as time takes it’s toll – it becomes increasingly
unmanageable.
It is divided up into three
classes although, like Parkinson’s Disease, each person has different
symptoms and complaints. Each category has some common symptoms but
symptoms are not strictly found only in that category.
The three categories are:
- Shy Drager Syndrome (SDS)
- Striatal Nigral Degeneration (SND)
- Olivoponticerebellar Atrophy (OPCA)
Shy Drager Syndrome primarily
starts as an autonomic system disease. The primary symptom is postural
hypotension. Postural Hypotension is a drastic decrease in blood pressure
when moving to a standing or sitting position. The change in pressure can
cause dizziness and blackouts. Other symptoms include generalized
weakness, double vision and/or other vision disturbances, impairment of
speech, sensory changes, difficulties with breathing and swallowing,
irregularities in heart beat, inability to sweat, and diarrhea as well as
symptoms seen in the other two categories. Shy-Drager is often difficult
to treat because of the fluctuations in blood pressure. The general
treatment course is aimed at controlling symptoms. To relieve low blood
pressure, dietary increases of salt and fluid may be beneficial.
Medications to elevate blood pressure such as corticosteroids may cause
side effects and should be carefully monitored by a physician.
Striatal Nigral Degeneration is a
sporadic, middle-aged onset degenerative disease of the nervous system.
Those who have SND (Striatal Nigral Degeneration) are the easiest to
misdiagnose as Parkinson's Disease even though tremor at rest is somewhat
uncommon in SND. Signs include any of the Parkinson’s symptoms. One study
reports that 5 out of 10 people diagnosed with SDS were originally
diagnosed with PD. Treatment with Parkinson’s meds is moderately
successful due to the fact that these diseases affect more than one area
of the brain. It is not uncommon to see normal dopamine levels. By using
dopamanergic agents, the receptors are flooded so that a positive result
may occur. As with the other two categories, symptoms are shared.
Olivopontocerebellar Atrophy or
OPCA is a progressive degeneration of the cerebellum, the pons, and the
inferior olives of the brain. This degeneration results in a variety of
ataxias that can vary in age of onset and severity. Symptoms primarily
include balance, coordination and assorted speech difficulties. Symptoms
of the other categories may be present in this category and usually appear
as the disease advances. Symptoms are treated with a variety of
medication as well as physical therapy.
Medications, surgical procedures
and any other treatment are not cures and the results vary from individual
and category. For example, DBS would not be useful except for those in
early stages of SND. Medications used to raise blood pressure would be
useful primarily in those with SDS. It is a matter of making the patient
comfortable as long as possible. Pain medications are also used to
provide comfort but doses can increase rapidly.
This is a brief synopsis of this
disease. Lifespan can be as short as five years or as long as twenty
years depending on rate of progression, age of onset as well as other
factors. Death is usually a result of pneumonia.
On a personal note, I was
diagnosed originally with Parkinson’s two years ago. After my health
declined at an extraordinarily uncommon rate and the occurrence of
symptoms such as eye disturbances, incontinence, I was rediagnosed with
MSA. At that time, my wife was told I had probably 3 to 5 years to live
and my career as a pharmacist ended. Each day is a wonderful event in our
family and we make the best of all things. I am now in bed about 18 hours
a day and use a quad cane to get around the house and a wheelchair in
public. A hospital bed, commode, and Ensure have become elements of my
new bedroom in the dining room. Instead of feeling sad, it has brought a
renewed sense of family and community to our house. We try not to think
in terms of years left although the rapid rate of progression is a
constant reminder. We keep busy, I try to help others through PLWP and my
web site, and we thank God every day for what we have and do not blame Him
for what we don’t because there are always people worse off than we are.
Tim Foley
For
more information, contact me at
tpfrph@yahoo.com or visit my web site at
http://msainfo.tripod.com. Another great online source of patients
and caregivers can be found at
http://groups.yahoo.com/group/shydrager .