Confused about OPCA/SDS/SND/MSA? Explanation here.

[Guest guest]

MSA = Multiple System Atrophy

MSA is ONE DISEASE with 3 forms:

1. OPCA = Sporadic Olivopontocerebellar atrophy (see more notes about OPCA

at the end)

2. SDS = Shy-Drager Syndrome

3. SND = Striatonigral Degeneration

The reason there are 3 forms is that years ago all doctors had to go on was

symptoms. If a patient had loss of balance(ataxia) and slurred speech then

they knew they had nerve degeneration in the cerebellum, olives and pons

(areas of the brain). They called this Olivopontocerebellar atrophy(OPCA).

If a patient had low blood pressure (orthostatic hypotension) they knew that

they had nerve degeneration in the autonomic nervous system. They called

this Shy-Drager Syndrome(SDS).. named for Dr. Milton Shy and Dr. Glen Drager

who first wrote about this disorder in 1960.

If a patient had tremors, slowness of movement and other Parkinson-like

symptoms they knew that they had nerve degeneration is the striatonigral

pathway of the brain. They called this Striatonigral Degeneration(SND).

Doctors once thought that these 3 disorders were all SEPARATE diseases.

They now know that THIS IS NOT THE CASE. Autopsy evidence has shown that

there appears to be a common underlying cause in all three disorders so they

came up with a NEW NAME to include all 3.... MULTIPLE SYSTEM ATROPHY. Many

doctors have been slow to adopt this new terminology so continue to use the

old terms OPCA, SDS and SND when diagnosing.

In 1995, a consensus statement was approved by the American Autonomic

Society and the American Academy of Neurology to clarify the definitions of

MSA. Multiple system atrophy is now defined as " a sporadic, progressive,

adult-onset disorder characterized by autonomic dysfunction, parkinsonism,

and ataxia in any combination. "

The term " sporadic " means that the disease is NOT hereditary.

NOTES about OPCA:

-----------------------

OPCA is a more confusing diagnosis to interpret because there are many

different disorders that show the symptom of loss of balance (ataxia). Dr.

Sid Gilman at the University of Michigan has published some recent research

that suggests that only about 1/4 of all cases diagnosed as OPCA actually

belong under the category of Multiple System Atrophy(see article abstract

below). If you'd like to know more about sporadic OPCA please write and ask

me for more information. My mother-in-law had this diagnosis... in her

case it appeared to clearly progress to MSA within 5 years but I do know of

many others who have not followed this rapid progression and therefore

likely do not have a form of sporadic OPCA considered to fall under the MSA

umbrella.

Regards,

Pam

pbower@...

---

Neurology 2000 Aug 22;55(4):527-32

Evolution of sporadic olivopontocerebellar atrophy into multiple system

atrophy.

Gilman S, Little R, Johanns J, Heumann M, Kluin KJ, Junck L, Koeppe RA, An

H.

Department of Neurology, University of Michigan, Ann Arbor, 48109-0316,USA.

OBJECTIVE: To determine the percentage of sporadic olivopontocerebellar

atrophy (sOPCA) patients who later develop multiple system atrophy (MSA).

METHODS: Observations of the course of 51 sOPCA patients 20 years of age or

older initially evaluated in an ataxia clinic over 14 years and followed at

3- to 6-month intervals for 3 months to 10 years (median 2.5 years,

interquartile range 5 months to 4 years). RESULTS: Seventeen patients

evolved to develop MSA, whereas the remaining 34 manifested only

progressively worsening cerebellar ataxia. The features of the MSA cases

included autonomic failure and parkinsonism in 10 patients, autonomic

failure without parkinsonism in six, and parkinsonism without autonomic

failure in one. Using survival analysis methods, the authors estimated that

24% of subjects in this population will evolve to MSA within 5 years of the

onset of sOPCA symptoms (95% CI 10% to 36%). An older age at onset of

symptoms and a shorter time from onset of symptoms to first presentation in

a neurology specialty clinic were both highly predictive of evolution to

MSA. Six of the 17 patients who evolved to MSA died 4 months to 5 years

after they had met diagnostic criteria for MSA. The estimated median

survival time from time of transition was 3.5 years. In contrast, death

occurred in only one of the 34 patients with sOPCA who did not evolve to

MSA. Autopsy examination of all six patients with MSA who died confirmed the

diagnosis. CONCLUSIONS: Approximately one-fourth of sporadic

olivopontocerebellar atrophy patients will evolve to multiple system atrophy

within 5 years, and this transition carries a poor prognosis for survival.

Older age at onset of ataxia and earlier presentation in a neurologic

specialty clinic predicted transition to MSA.

PMID: 10953186 [PubMed - indexed for MEDLINE]

[Guest guest]

Hi Janet,

I came up with this Neopolitan ice cream analogy awhile ago to explain

Multiple System Atrophy and it's 3 flavours. (Neapolitan ice cream is that

3 flavoured ice cream with strawberry, chocolate and vanilla all mixed

together.)

Think of it this way:

Shy-Drager Sydrome(SDS) is the strawberry flavour of MSA

Olivoponto cerebellar atrophy (OPCA) is the vanilla flavour of MSA

Striatonigral Degeneration (SND) is the chocoloate flavour of MSA

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.

Whether someone is told they have SDS or SND or OPCA they all have one scoop

of Neapolitan ice cream in their dish. They all have Multiple System

Atrophy.

If their symptoms are mainly ataxia they have mostly vanilla ice cream in

their dish (OPCA). If their symptoms are mainly Orthostatic hypotension

they have mostly strawberry ice cream in their dish(SDS). If their symptoms

are mainly tremors and slowness of movement they have mostly

chocolate ice cream in their dish.(SND) If they have symptoms of all three

then they might have pretty equal amounts of chocolate, strawberry and

vanilla in their dish.

One thing about MSA though is that over time the amounts of the flavours may

seem to change spontaneously and those who used to have just a little

strawberry flavour or SDS symptoms may develop more of them... in fact each

of the flavours may become more pronounced as time goes on... but this will

vary from person to person.

I hope this helps you understand MSA a bit better.

Take care,

Pam

> Hi Pam,

>

> You sent an email explaining the difference between Shy-Drager and the

> other Parkinson's Plus diseases.

> I'm somewhat confused - - do some people just have one of the disease

> types and never develop the others?

> or does it adventually progress to the point where a person has all three.

> And, would an MRI be able to show that you had MSA in the early stages?

>

> Thanks for your help.

>

> Janet

>

[Guest guest]

Pam, You are very creative !!!! Cheers - waverley>>> pbower@... 06/06/01 09:01PM >>> Hi Janet, I came up with this Neopolitan ice cream analogy awhile ago to explain Multiple System Atrophy and it's 3 flavours. (Neapolitan ice cream is that 3 flavoured ice cream with strawberry, chocolate and vanilla all mixed together.) Think of it this way: Shy-Drager Sydrome(SDS) is the strawberry flavour of MSA Olivoponto cerebellar atrophy (OPCA) is the vanilla flavour of MSA Striatonigral Degeneration (SND) is the chocoloate flavour of MSA 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. Whether someone is told they have SDS or SND or OPCA they all have one scoop of Neapolitan ice cream in their dish. They all have Multiple System Atrophy. If their symptoms are mainly ataxia they have mostly vanilla ice cream in their dish (OPCA). If their symptoms are mainly Orthostatic hypotension they have mostly strawberry ice cream in their dish(SDS). If their symptoms are mainly tremors and slowness of movement they have mostly chocolate ice cream in their dish.(SND) If they have symptoms of all three then they might have pretty equal amounts of chocolate, strawberry and vanilla in their dish. One thing about MSA though is that over time the amounts of the flavours may seem to change spontaneously and those who used to have just a little strawberry flavour or SDS symptoms may develop more of them... in fact each of the flavours may become more pronounced as time goes on... but this will vary from person to person. I hope this helps you understand MSA a bit better. Take care, Pam > Hi Pam, > > You sent an email explaining the difference between Shy-Drager and the > other Parkinson's Plus diseases. > I'm somewhat confused - - do some people just have one of the disease > types and never develop the others? > or does it adventually progress to the point where a person has all three. > And, would an MRI be able to show that you had MSA in the early stages? > > Thanks for your help. > > Janet >