Have been diagnosed with a very
rare neurological disorder
called the stiff person syndrome.
Which affects something like.
One in a million. Céline
Dion revealing her diagnosis
on Instagram this morning.
The Grammy winning singer had to postpone
and cancel some upcoming tour dates.
She has what she just called
stiff Person syndrome,
which causes sudden severe muscle spasms.
She says she has no choice but
to concentrate on her health,
saying the condition has affected
every aspect of her daily life.
Seeing Céline Dion had rescheduled
her spring 2023 shows to 2024,
she cancelled 8.
Show scheduled for summer of 2023.
Well,
joining us now to talk about
this rare disease, our Nine News
medical expert Doctor Paul Coley.
It is rare, one in a million.
But you actually have seen a case of this.
I've seen one single case in my
entire career in medical school.
It was so dramatic to see this because
this is not just muscle spasms every day,
muscle spasms that most of us get.
This is an inability to move your muscles.
Because of that stiffness or that spasm.
You could, you know,
not be able to walk.
You could fall over if you're standing.
And sometimes these muscle spasms are
actually triggered by environmental stimuli.
Like a a a sound could trigger the
muscle spasm or you know stress having
a lot of anxiety could trigger it
and it could be a small spasm,
1 area or is it does your whole body
spasm or how how does it portray itself.
So it kind of starts gradually
and and essentially what happens
is most classically present with
lower back muscle spasms and leg
muscle spasms that start out maybe
just like back pain or leg pain but
then as the disease progresses it
can start to become more severe,
can be asymmetric.
So it involves one side more than
the other and it can leave you
with an inability to use that.
Muscle groups. So for example,
if your leg muscles are involved,
you could have trouble walking
or standing with Celine.
I would worry obviously about other
types of muscles being involved as well,
you know,
for her performance and she's
got her vocal cords,
she even mentioned in some state.
When you bring up all this though,
it makes me say, well,
if it starts like as a little
spasm like most of us have,
it's got to be really hard to diagnose.
It is hard to diagnose,
but we're getting better at it.
So essentially what we think
causes it is an autoimmune
reaction and the reason we think.
That is because a,
it travels with other autoimmune diseases,
most commonly diabetes or your immune
systems kind of attacking your own body.
In this particular case that the
chemicals that are involved in turning
the volume up and down on your muscles,
on your motor neurons basically
that control your muscles.
But B, you can have antibody.
So 60 to 80% of people who have this
condition have an anti gad antibody
that's positive in their spinal fluid.
And I wonder if that's what
the case was for Selena,
is that the test? Limit lead to know
that you have this syndrome and not
just a terrible case of of spasms.
Yeah, I think that's probably
the test that would sort of
make it most likely to have it.
Now some people don't have this
antibody have a different one,
breast cancer runs with it too,
but it's really a a conglomeration of
all the symptoms put together that
helps to diagnose and determine it.
And what we're seeing is that used
to be called stiff Man syndrome.
They actually ended up changing the
name because we're seeing it more in
women than we see in men and that's
also something that's poorly understood,
we know that.
Autoimmune diseases in general are more
common in women than they are in men.
This doesn't mean you're
predisposed to anything that we
would think of as more severe,
like ALS or Ms or something that could.
Is it a signal that something
either has happened or is going
to happen later in your body?
No, but it's a great question because it
often gets misdiagnosed as ALS, Parkinson's,
or some other neurological condition.
And as you can imagine,
the treatments of prognosis and and all could
be very variable depending on what it is.
But this is really that
neurotransmitter is not working.
To modulate your motor neurons and so they're
firing when they shouldn't be firing,
which causes this muscles to go into spasm,
inability to use them. So if
it's that rare, the treatment must
be really hard too because like what
works if you have one in a million
how you going to find out what works
really hard so it's not able to be cured.
It's it's usually progressive
so people get worse over time.
So essentially we just sort of
treat the symptoms and the symptoms
of muscle spasm are treated
with you know antispasmodic so.
Muscle relaxants,
you can also use medications like Valium
or Ativan because those medicines help
that neurotransmitter be overexpressed.
So they modulate that.
Some anti seizure medications
work for it as well.
We're now studying,
you know, IVIG,
which is a way to turn down
the immune system,
kind of change the volume
on the immune system and
see if that helps to control
the IVIG. Tell tell me what
that what are you talking
about intravenous immunoglobulin.
So it's giving you a bunch of antibodies
that turn off your immune system.
It's a way to nonspecifically.
Turn off the immune system and
prevent your immune system from
making antibodies against,
you know, these neurotransmitters.
So if it's not at at present curable,
is it preventable? I mean,
if you get more iron in your
diet or you eat asaib berries or
whatever the the magic potion is,
is there anything that might help you not get
it? I wish we knew.
We just don't know because it's so rare,
it's so poorly studied,
and we don't really completely
understand the pathophysiology
because some people will just have
one set of motor neurons involved.
In other words,
just your back or just your leg and
other people will have their brains.
Them, and really their entire kind
of systemic motor neurons involved
how devastating this diagnosis for
her and for anyone with this so.
OK. Thanks for explaining
that though, Doctor Cole.
I think we learned a lot.
I mean, never heard of it
ever before until today.
Well, one in a million and and
she has it and you've seen one.
So that that also is one in
a million that that that both
of you are familiar with it.
Sadly for her, it's something
she's gonna have to deal with.
Doctor Powell Cola,
good to see as always. Thanks.