Multiple System Atrophy falls
into the category of Atypical Parkinsonism, or Parkinson plus syndrome, a group
of diseases linked to a lack of dopamine in the brain. Dopamine controls
movement. While Parkinson’s is the most common Parkinsonism, approximately 20
percent of people will be diagnosed with another Parkinson’s-like condition.
What is Multiple System Atrophy?
Multiple System Atrophy (MSA)* is a progressive
brain disorder caused by loss of nerve cells in specific areas of the
brain. This loss causes problems with movement, balance and autonomic functions
of the body. (Autonomic functions are body functions that occur automatically,
such as bladder control.)
The term MSA stands for:
Multiple — many
System — brain structures
that control different functions
Atrophy — cell shrinkage or damage
Which parts of the brain are affected in Multiple
System Atrophy?
In MSA, cells are damaged in
different areas of the brain which control a variety of body functions. The
three areas affected are the basal ganglia, cerebellum and brain stem.
In MSA, brain cells in the affected
areas 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 autonomic control centres of the brain that confirms a
diagnosis of MSA.
What are the symptoms of MSA?
The
symptoms of MSA can be put into three groups. Having a diagnosis of MSA means
you have a combination of symptoms from at least two of the groups:
Parkinson's symptoms – relating to slowness and stiffness of movement
•
feeling slow and stiff when moving
•
difficulty turning in bed
•
difficulty starting to move
•
difficulty fastening buttons on a shirt or
blouse.
Cerebellar symptoms – relating toco-ordinating movement and balance
•
feeling clumsy, dropping things
•
feeling unsteady in crowds
•
inability to balance without support
•
difficulty writing
•
slurred speech
Autonomic symptoms – relating to automatic body functions
•
bladder problems
•
erectile dysfunction
•
dizziness or fainting (blood pressure
problems)
•
constipation
•
cold hands and feet
•
inability to sweat
Other symptoms may include:
•
weakness in arms and legs
•
heightened emotional response
laughing or crying disproportionate to
the
situation)
•
restless sleep
•
nightmares
•
noisy breathing during the day, snoring
at night
• unintentional
sighing
•
swallowing problems, difficulty chewing,
choking episodes
•
weak, quiet voice
• cognitive
problems, slowness in
thinking, difficulty with multi-tasking
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What causes Multiple System Atrophy?
It
is unclear why the cells become damaged in MSA. It does not appear to be
inherited, although some research suggests there may be a predisposition to
MSA due to the individual’s genetic make-up. What triggers the damage is
unknown. Environmental toxins or a history of trauma is a focus of ongoing
research. MSA is not infectious or contagious.
How is someone diagnosed with Multiple System Atrophy?
Due
to the variety of symptoms present in MSA, it is often difficult, at first,
to differentiate it from Parkinson’s disease. Sometimes, it can take years
before a distinction can be made. Generally,
MSA has a more rapid decline, no tremor, early autonomic symptoms, and motor
symptoms on both sides of the body rather than one side. If someone responds poorly
to medications used for treatment of Parkinson’s disease, this may be a clue
that MSA is present. Also, if you still have your sense of smell, this can
distinguish MSA from Parkinson’s because loss of smell is common in people
with Parkinson’s and not as common in MSA.
An
assessment by a movement disorder
specialist
can determine if you have MSA. Included in this assessment would be a complete
medical history, a physical exam and tests, including brain imaging.
What is the progression of Multiple System Atrophy?
MSA
is a progressive brain disease and everyone will experience MSA differently.
However, the symptoms will change. In MSA, the changes occur more rapidly
than with Parkinson’s. You will need more help to care for yourself as symptoms
impact your daily life. Lowered blood pressure can cause fainting and falls.
Loss of co-ordination, slowed
movements, and rigidity can interfere with
activities of daily living.
How is Multiple System Atrophy treated?
Currently,
there is no treatment to slow the progression of the disease. The complex
nature of MSA means you should see a neurologist who specializes in movement
disorders. It is important to look at and treat each symptom separately in
order to maintain daily activities and quality of life. Often a
multidisciplinary team, including a neurologist, social worker, speech
language pathologist, physiotherapist, urologist, clinical nurse and family
doctor will be involved
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This is a good analogy by Pam Bower explaining MSA.
"WHAT IS MULTIPLE SYSTEM ATROPHY(MSA)? I thought of this analogy many years ago to describe MSA, I've modified it a little bit: MSA is like the three flavoured Neapolitan ice cream. Everyone with MSA gets a scoop of the MSA ice cream just as everyone at a birthday party gets a scoop of Neopolitan ice cream in their dish. If you were at the party and looked at your dish and at the dish of the person next to you you would notice that there is not the exact same amount of vanilla, chocolate and strawberry in both of the dishes. In someone with Multiple System Atrophy, if their symptoms are mainly CEREBELLAR like ataxia (loss of balance), slurred speech or difficulty swallowing they have mostly vanilla ice cream in their dish . If their symptoms are mainly PARKINSONISM like tremors and slowness of movement they have mostly chocolate ice cream in their dish. If their symptoms are mainly AUTONOMIC like Orthostatic hypotension or urinary incontinence or urinary retention or sexual dysfunction they have mostly strawberry ice cream in their dish. Everyone needs to have some strawberry symptoms to have possible MSA. On top of this they need to have some vanilla and some chocolate symptoms too. Note that they may have a whole lot of vanilla and a tiny bit of chocolate. If so they are called MSA-C (cerebellar). If they have a whole lot of chocolate and a tiny bit of vanilla they are called MSA-P (parkinsonism). People with MSA have a changing bowl of ice cream though ... their vanilla and chocolate and strawberry amounts increase over time as they develop more symptoms from the cerebellar or the parkinsons or the autonomic sides. ... but this will vary from person to person. This is because the disease is spreading throughout the brain. It does not spread exactly the same way in every person. I hope this helps you understand Multiple System Atrophy a bit better. "
Below are some various links - in no particular order of importance. Admittedly its quite an intimidating list - but then MSA is an intimidating illness. If you are a patient - this information can be scary and JUST TOO MUCH - take your time and don't try to read too much too soon. You have so much to deal with and need to take your time to digest.
Reflecting back on Sonja's journey - I recall everything being so overwhelming in the beginning. First of all she went to more than one Specialist. Only when the diagnosis were independently and repeatedly given - could the journey to acceptance begin. Sonja opened all the doors she could find to deal with every obstacle as it came her way - sometimes fortunately anticipating the next stage - for instance when she acknowledged that the steps leading in and out of the house needed to be replaced with ramps. Her bathroom was a big project - she got a person in who specialises in the design and adaptation of bathrooms for disabled people. The builder was given the specifications by her and the toilet, basin, the taps, the handrails etc, the wheelchair shower chair, the access to the wheel-in shower, etc - all contributed immensely to less frustrating bathroom routines.
Looking back also - it was when Sonja allowed herself to open the door to other patients on the social media - that her life with this debilitating disease - became purposeful.
You are reading this for a reason. May you find comfort in the posts and journey shared in this blog. May you too be reminded that your life has a purpose - even in these dark moments. Try not to dwell too much on the grim and dreary posts. The MSA Coalition and other MSA organisations are fantastic. Connect with them.
My sincere wishes to you and your family.
Karin
http://www.msainsouthafricawithsonja.blogspot.com/p/msa-in-nutshell.html
http://www.msainsouthafricawithsonja.blogspot.com/
https://www.facebook.com/Miracles.for.MSA?fref=ts&hc_location=ufi
https://www.facebook.com/groups/WorldMSADay/?hc_location=ufi
http://sonjasegriffels.blogspot.com/
http://www.msatrust.org.uk/
http://www.msaawareness.org/?page_id=122
https://www.facebook.com/groups/msaangels/?ref=br_rs
https://www.facebook.com/groups/MSANewsGroup/?ref=br_rs
https://www.facebook.com/groups/MSABuddies/?ref=br_rs
http://www.msatrust.org.uk/living-with-msa/how-the-trust-can-help-you/fact-sheets-download/
https://www.facebook.com/groups/MSANewsGroup/?hc_location=ufi
https://www.facebook.com/pages/Health-Insight/133520186743182?hc_location=ufi
https://www.facebook.com/rarediseaseday?fref=n&hc_location=ufi
http://www.healthinsight.co.za/www/
https://www.facebook.com/PatientsLikeMe?fref=nf&hc_location=ufi
https://www.facebook.com/pages/Multiple-System-Atrophy-Trust/182824945086605?hc_location=ufi
https://www.facebook.com/NationalOrganizationforRareDisorders?fref=nf&hc_location=ufi
https://www.facebook.com/rareconnect?fref=nf&hc_location=ufi
https://www.facebook.com/pages/Rare-Diseases-South-Africa/431020690310763?hc_location=ufi
https://www.facebook.com/DysautonomiaInternational?fref=nf&hc_location=ufi
https://www.facebook.com/inspirationalme?fref=nf&hc_location=ufi
https://www.facebook.com/DefeatMSAJGFFoundation?fref=nf&hc_location=ufi
https://www.facebook.com/michaeljfoxfoundation/posts/10153293298003540?hc_location=ufi
https://www.facebook.com/pages/Rare-Diseases-South-Africa/431020690310763?hc_location=ufi
https://www.facebook.com/ProfGregorKWenning?fref=nf&hc_location=ufi
A pdf file to download - also a handy link to send to your medical practitioner / carer / hospice