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MSA - Multiple System Atrophy
Multiple System Atrophy (MSA) is a progressive neurological disorder that affects adult men and women. MSA is caused by degeneration of nerve cells in specific areas of the brain. This cell degeneration causes problems with movement, balance and automatic functions of the body such as bladder control.
MSA is a rare neurological disorder characterized by a combination of parkinsonism, cerebellar and pyramidal signs, and autonomic dysfunction, affecting movement, blood pressure, and other body functions; hence the label "multiple system" atrophy. Its cause is unknown. Its symptoms vary in many ways from person to person.
Three different diseases were initially described to take in this range of symptoms:
Shy-Dragger syndrome, striatonigral degeneration, and olivopontocerebellar atrophy. In Shy-Dragger syndrome mainly involves the autonomic system, the body system that regulates blood pressure, urinary function, and other functions not involving conscious control.
Striatonigral degeneration causes Parkinsonian symptoms such as slowed movement and rigidity. Olivopontocerebellar atrophy (OPCA) principally affects balance, coordination and speech. The average age for the onset of this disease in 54 years, and it occurs in people between the ages of 50 and 99 years in 1 out of 33,000 people. Parkinsonism is the most common initial sign and eventually develops in about 90 % of cases.
Sporadic OPCA evolves into MSA in roughly 25% of cases within 5 years. Sometimes there is nystagamus - eye problem. Autonomic dysfunction includes impotence, postural hypotension, with syncope (a brief loss of consciousness due to a sudden fall in blood pressure), urinary incontinence and retention, and fecal incontinence. About 80% are disabled within 5 years of the onset of the motor symptoms. The mean survival is roughly 6 years.
I think I have Sporadic OPCA with MSA.
How M.S.A. is treated:
• to treat slowness and rigidity: Levodopa and dopamine agonists
• to raise blood pressure: fludocortisone and midorine.
**(CAVEAT: Be sure you take only those medications which are prescribed by a physician.)
How common is MSA?
Until recently MSA was thought to be a very rare disorder. As we learn more about the condition, it becomes easier to recognize and diagnose. Recent research suggests that MSA affects 5 people per 100,000 in the UK. This means that there may be up to several thousand people with MSA at any time in the UK.
Who has MSA?
MSA usually starts between the ages of 50-60 years, although it can affect people younger and older than this. MSA does not appear to be hereditary and is not infectious or contagious. It is a sporadic disorder that occurs at random.
What does MSA mean?
The term Multiple System Atrophy was first used in 1969, although it has been called many things including; olivopontocerebellar atrophy, striatonagral degeneration, Parkinson's plus and Shy-Drager Syndrome. The confusion caused by this variety of names led to an international consensus by medical experts in 1996 to use the term Multiple System Atrophy (MSA).
Broken down MSA stands for:
Multiple - many
System - brain structures that control different functions
Atrophy - cell shrinkage or damage
This means that cells are damaged in different areas of the brain that control different body functions. The three areas affected are the basal ganglia, cerebellum and brain stem. These areas are responsible for movement, balance and automatic body functions like bladder control
How are the nerve cells in the brain damaged in MSA?
Nerve cells in the affected areas of the brain shrink (atrophy). This can sometimes be seen on MRI scans. When brain tissue is examined under a microscope, structures called glial inclusion bodies can be seen; they contain a protein called alpha-synuclein. It is the presence of these inclusion bodies in the movement, balance and automatic control centers of the brain that confirms a diagnosis of MSA.
Why do nerve cells become damaged?
It is still unclear as to exactly why the cells become damaged in MSA. MSA is not contagious. It does not appear to be inherited, although there may be a predisposition within the genetic make-up of an individual for cells to become damaged. What triggers the damage process to begin is unknown. This is a focus of ongoing research.
What are the first signs of MSA?
For men, the first sign is often erectile dysfunction (unable to achieve or sustain an erection). Both men and women often have early bladder problems: urgency, frequency, incomplete bladder emptying or an inability to pass urine (retention). These problems may sometimes be incorrectly attributed to the ageing process or to prostrate disease in men.
Other early problems can be feeling stiff and slow and finding movement difficult, feeling dizzy when standing up, fainting, difficulty turning in bed, and changes in writing. Some people become clumsy or unsteady when walking.
However these early symptoms could also be due to a range of other diseases which need to be excluded before a diagnosis of MSA can be made.
What happens next?
MSA is a progressive disease - this means that your symptoms will change over a period of time and you will need more help to care for yourself. The speed of these changes is difficult to predict as people with MSA experience the disease differently. Some people feel they cope better when they know what lies ahead. You can discuss the future with your specialist or the Sarah Matheson Trust (SMT) nurses.
My MSA:
I have had MSA from the 'get-go'. I had constipation since 1958 and I was diagnosed in 2002 with this wretched Ataxia. So, I’ve been constipated for 48 years. That has been the case, too since my diagnosis, except for a few months two years ago.
During this time, I would have episodes of having to have a bowel movement, or of diarrhea, and I was afraid to go out, or commit myself to anything, for fear of having an episode. If I felt I had to 'go', it was too late! I would soil myself, because the sphincter muscles were non-functional. I could not depend on the usual signs of having to 'go'.
At the first rumble of my stomach, or the first cramp, I would get myself to the bathroom. I could neither delay having a 'movement' or could I hasten it. I had no control over the sphincter, one way or another. In the past, probably starting in 1994, I would have trouble having a movement. The waste would come out with no form but would ooze out, like wet mud, when I grunted.
It was then that I knew something was amiss. I couldn’t have a 'movement' but it was not because of constipation but because the nerves and muscles down there didn’t work. I could go for days without having a 'movement'. I didn’t feel any need to have one, even though waste was backed up near the sphincter. Neither the muscles or the nerves of the sphincter were regulated ( I didn’t feel as though I had to go). Whenever I felt like I was overdue for a 'movement', I would go to the bathroom and try to have to one. Usually, the waste was sitting there, ready to be evacuated. I say all this to indicate that I have had this condition for sometime. I’ve been impotent since the summer of 1993. Not only do I have erectile difficulty, but I have no libido. This is an early symptom of MSA.
Another symptom that is early is low blood pressure. I was hospitalized for two weeks in late June 2003 for low blood pressure. I had got an infection in my bladder. In my weakened state, I almost fainted during two successive nights. In both cases, I was in the bathroom. I crumpled to the floor and could not get up, but slithered along the floor to my bed. It took from 20 to 30 minutes. The next day, I was taken to hospital. My blood pressure was 60/40! I should have been dead! I came away with a prescription for AMATINE.
In March 2003, I had a 'supra-pubic' catheter put in, after some months with a regular one. The first one leaked around the outside, as though the catheter were too small.I immediately had a new problem: I could not find a 'holster' for the 'leg bag'. I had only the two straps to support the leg bag, I am allergic to Latex. The stretchy-y straps pull the hair on the leg, the full bag slipped down the leg. So I invented a 'holster'. I am trying now to get it patented. When the leg bag is properly fixed in place, the one wearing it does not know that he has a catheter installed.
In November 2004, I fainted after leaving the breakfast table. I must have been out for about fifteen minutes because when I woke up, I was on a gurney with the Emergency people working over me and heading for the hospital. That day, I started using a 'walker' (Nov., 2004). I stared using a wheelchair in June 2005.
OTHER SYMPTOMS I HAVE WITH MSA
Swelling of legs, trouble swallowing, inability to write, eyes do not focus (double vision) and dry mouth, if not careful with medication timing.
PROGNOSIS
It is not encouraging. It is said that people who have M.S.A. have a longevity of ten years after diagnosis. I was diagnosed with degeneration of the Cerebellum in May 2003….You can do math! But a gentleman lived with it for 25 years. But the telling part is in your answer to this: Why do you wish to live with this for a long life?
TREATMENT
There is no cure, but the symptoms can, however temporary these are, be assuaged. They are temporary in the sense that there are so many symptoms, that you become preoccupied with a new, or the onset of a new symptom and its treatment. It is not that the symptoms disappear. The symptoms have not been overcome, mostly because the doctors and researchers do not know the cause of MSA.
This is written so that MSA can have a human face. If you have MSA, or think you have MSA, be sure a professional make this diagnosis. The professionals often know very little about this, because it is so rare.
It is then you can start your own research about your own kind of MSA. You do your own research based on your own symptoms. This is where your are the authority.
National Ataxia Foundation: www.ataxia.org
*Web Site: NEUROLOGY, Northwestern University Medical School, Multiple System Atrophy, Hain, M.D. Timothy C., Jan 2001.
**Web Site: WE MOVE, Worldwide Education and Awareness of Movement Disorders. Overview o Multiple System Atrophy. Jan 20, 2005, N.Y. +ibid.
**We Move 2004- Overview if Multiple System Atrophy, January 20, 2005. P.1