Episode Transcript
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For the past few days, you have been experiencing a tingling sensation in your toes, which you
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initially attributed to being overly tired and the frigid winter season.
But when a pain erupted like electric shocks through your legs, stealing away your sleep
and leaving you restless, that assumption began to crumble.
This morning, when you got out of bed, you struggled to walk and noticed that your hands
were also starting to feel tingly.
Concerned about these unsettling symptoms, you decided to book an appointment with a
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doctor.
[Intro Music]
Hello and welcome to Anatomy of Illness.
Today's episode is about Guillain-Barré Syndrome.
Before we get into the condition, we are going to start with the history.
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So why do we know Guillain-Barré Syndrome exists?
We begin in 1859 with a French physician and medical researcher by the name of Jean-Baptiste
Octave Landry.
He reported on something he described as acute ascending paralysis.
In his publication, he wrote, “The sensory and motor Systems may be equally affected. However the main problem is usually a motor disorder characterised by a gradual diminution of muscle strength with flaccid limbs without contractures. When paralysis reaches maximum intensity, the danger of asphyxia is always imminent. However in eight out of ten cases death was avoided. When there is a reversal of paralysis,
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the recovery period involves a phenomena opposite to those indicated in
the development period.
Patients either recover quickly or the disease becomes chronic with slow improvement.”
This publication initially described the features of Guillain-Barré Syndrome and would lead
to the use of Landry's ascending paralysis as a way to describe the condition.
This helped to differentiate the condition from polio.
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Now we move to 1891 with Walter Essex Wynter.
In this year, he published the first account of lumbar cerebrospinal fluid sampling using
a cut down technique.
Also in the year 1891, Quinke developed the lumbar puncture technique for testing cerebrospinal fluid.
Now we move into 1916.
We are watching three French army physicians at the neurological center of the French 6th
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Army.
They are discussing the case of two paralyzed French soldiers.
But who were these three French physicians?
Guillain, Barré and Strohl.
Strohl is kind of like the third Hemsworth brother.
In this, you never knew he existed, but he is there.
Anyway, back to their discussion.
So they were discussing the tendon reflexes of these two soldiers and also what is in
their cerebrospinal fluid, also known as CSF.
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In the CSF, they found high levels of proteins.
This was different from other causes of flaccid paralysis, such as polio and syphilis.
This led to it being described as its own entity, its own condition, which is where
the name Guillain-Barré Syndrome was born.
Initially it was called Landry-Guillain-Barré-Strohl Syndrome because obviously Landry's initial
description and Strohl had been rather influential and helpful in describing the cases of these
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two soldiers.
He had taken the electrographical recordings and also characterized the loss of tendon
reflexes.
But hey, it was a group project.
The name of the condition Landry-Guillain-Barré-Strohl Syndrome being an absolute mouthful led to
the 1927 decision to shorten it.
Because Guillain and Barré were highly regarded neurologists after the war and also because
Guillain claimed it was a different condition from what Landry described, the name was shortened
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to Guillain-Barré Syndrome.
With this, there was much up for debate as Landry had not mentioned bladder involvement,
nor had he mentioned the cranial nerves.
Neither had Guillain, Barré or Strohl, but with time Guillain and Barré did accept these
as potential symptoms.
Due to all of this, there was much muddiness in the diagnostic waters.
In 1949, a case series of 50 Guillain-Barré Syndrome patients autopsies would be published
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by Haymaker and Kernohan.
In today's classifications, up to a quarter of these patients would not have fit the diagnostic
criteria.
However, this is one of the largest and most comprehensive pathological studies of Guillain-Barré
Syndrome.
In this study, they found several things.
These included nerve edema, which is swelling and fluid buildup during the first few days
after the onset of Guillain-Barré Syndrome.
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After this, there was swelling in the myelin sheaths, which are the fatty protective covers
that insulate the nerve fibres and irregularities in the nerve axons, which are the nerve fibres
that send communications out.
For those who had passed around the 9-day mark, they had lymphocytic infiltration and
macrophagic infiltration.
A few days later, there was also evidence of Schwann cell proliferation, so a massive
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increase in Schwann cells.
These cells are the cells that form the myelin sheath.
From this study, they created the basis of a pathological understanding of Guillain-Barré
Syndrome.
But they also proposed what would be the initial concept of what caused the condition.
They believed that the nerve destruction was not the initial cause but a reaction to an
injury instead.
Now we move to 1956, to Massachusetts, to meet the Canadian doctor Charles Miller Fisher.
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A little side note, Dr. Fisher is a really interesting and really influential person.
During the Second World War, he ended up in a prisoner of war camp.
As a prisoner of war, he ended up being a doctor for others at the camp during his three
and a half years there.
Anyway, moving on.
Why does he matter in the history of Guillain-Barré Syndrome?
Well, Dr. Fisher reported the cases of three patients and their case histories in a publication
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in the New England Journal of Medicine.
These three patients had some peculiar symptoms.
These were areflexia, ataxia, and ophthalmoplegia.
Areflexia being an absence of normal body reflexes.
So you know if a doctor has ever smacked you in the knee with a small hammer type thing
and your leg moved, that is a reflex.
Areflexia is when you don't do that.
Ataxia, this is impaired balance or coordination.
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Ophthalmoplegia, so this is paralysis or weakness of the eye muscles.
So the eye muscles can't move.
So you struggle to look around.
Interestingly enough, one of the cases that Dr. Fisher wrote about was actually what a
patient had described to him after the illness had occurred.
The patient had given him a detailed description of the event and also their clinical records,
leading him to draw conclusions and include them in the case studies.
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He called what affected these three patients an unusual variant of idiopathic polyneuritis.
He considered it to be an unusual and unique variant of Guillain-Barré Syndrome without
having an unusual and unique disturbance of peripheral neurons.
This work from Dr. Fisher would lead us to the understanding that Guillain-Barré Syndrome
was a spectrum of conditions and included several conditions including Bickerstaff Brainstem
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Encephalitis, Miller-Fisher Syndrome of course, and several others.
Speaking of spectrums, we have a spectrum of cool options for merch at merchanatomy.com
so you can stay warm in the winter or look cool in summer and even stay hydrated.
What actually causes Guillain-Barré Syndrome?
Well, we don't actually know.
However, about two-thirds of people with the condition prior to experiencing it have some
form of infection in the six weeks prior to Guillain-Barré developing.
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These can be respiratory or gastrointestinal illnesses.
What are some of the types of Guillain-Barré Syndrome?
Guillain-Barré Syndrome can be considered as a sort of umbrella term.
The main forms of Guillain-Barré Syndrome include acute inflammatory demyelinating polyradicular
neuropathy also known as AIDP.
So AIDP begins as a muscle weakness that starts in the lower part of the body and progresses
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upward.
This form is the most common form in North America and Europe.
Miller Fisher Syndrome. In this form paralysis actually starts in the eyes and this form is associated with an unsteady walk.
This form is less common in the US but is more common in Asia.
Acute motor axonal neuropathy which is AMAN and acute motor sensory axonal neuropathy
which is AMSAN.
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These are less common in the US but are more common in China, Japan and Mexico.
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How do we diagnose Guillain-Barré Syndrome?
Initially it can be hard to diagnose Guillain-Barré Syndrome especially when symptoms aren't
only just beginning.
With this the symptoms are similar to other conditions and also the symptoms can vary
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between person to person.
With this condition the diagnosis process begins with a thorough medical history and
physical examination.
After this there are several medical tests that may be recommended as part of the diagnostic
procedure.
These include spinal tap also known as a lumbar puncture.
This is where a needle is inserted into the spine and the cerebrospinal fluid is collected
from the lower back.
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This is then tested for changes that are normally observed in Guillain-Barré Syndrome.
Electromyography This is where thin needle electrodes are inserted
into the muscles and are used to measure the nerve activity.
Nerve conduction studies This is where electrodes are taped to the
skin above where the nerves are located.
A small shock is then passed through these electrodes to the nerves to measure the speed
of the nerve signals.
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These tests can help to provide a picture of what may be occurring.
We'll get right into the symptoms and presentation right after this little break.
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What symptoms might you have if you had Guillain-Barré Syndrome?
The symptoms you might have if you had Guillain-Barré Syndrome include a feeling of pins and needles
in the fingers, toes, ankles and wrists.
Weakness in the legs that spreads upwards into the upper body.
Severe pain This may feel achy or shooting or cramp like.
This may also feel worse at night.
You may also have an unsteady walk, not be able to walk or struggle to walk up the stairs.
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You can also have low or high blood pressure.
A rapid heart rate.
Trouble with breathing.
Trouble with bladder or bowel control.
You may also have trouble with facial movements including speaking, chewing or swallowing.
You may even have double vision or difficulty with moving the eyes.
The symptoms will vary depending on the form and every person's symptoms will be slightly
different.
What should a doctor be aware of when it comes to Guillain-Barré Syndrome?
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Some things a doctor should be aware of when it comes to Guillain-Barré Syndrome are
Trouble breathing With the condition, the paralysis can spread
from the lower limbs to the muscles that are used for breathing.
In up to 22% of cases of Guillain-Barré Syndrome, temporary assistance from a machine is needed
to help breathe during the first week in the hospital with the condition.
Blood clots People with Guillain-Barré Syndrome are at
an increased risk of blood clots when they are unable to move.
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This may lead to the use of blood thinners and specific support stockings for blood flow
until they are able to move independently.
Pressure sores or bed sores are another issue that can inadvertently be related to Guillain-Barré
Syndrome.
These sores can be caused by remaining in the same position for long periods of time,
which can occur with paralysis from Guillain-Barré Syndrome or other causes of paralysis.
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Pain. One third of people with Guillain-Barré
Syndrome experience nerve pain.
Trouble with bladder and bowel functions.
Some people with Guillain-Barré Syndrome may end up with sluggish or slow bladder and
bowel functions as a result of the condition.
Recovery In most cases, Guillain-Barré Syndrome is
not fatal.
In some cases, it can be.
When it comes to recovering from Guillain-Barré Syndrome, it can take up to several years.
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Most people, however, can walk within 6 months of experiencing symptoms.
However, some people who have had Guillain-Barré will experience long-lasting symptoms.
These can include weakness, numbness, and fatigue, residual numbness, or other sensations.
As I said, most people with the condition will recover completely.
However, some will experience some minor residual weakness, tingling, and numbness.
Heart and blood pressure problems With Guillain-Barré Syndrome, fluctuation
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in both heart rate and blood pressure are common during the illness.
Relapse A small amount of people who have had Guillain-Barré
Syndrome will experience the condition again, so they will relapse.
This can cause muscle weakness years after the initial symptoms stopped.
How do we treat Guillain-Barré Syndrome?
There is no cure for Guillain-Barré Syndrome.
The approach to this condition is using treatments that can help speed up the recovery, and also
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to use treatments that help to deal with the symptoms and side effects.
Treatments that help to speed up the recovery include
Plasma exchange. This is plasmapheresis.
Plasma is the liquid portion of the blood.
With plasma exchange, the blood comes out of the body.
I know it sounds sci-fi, but stick with me.
Plasma and your blood cells get separated.
Blood cells go back into the body, which makes more plasma, to replace the plasma we just
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got rid of.
The idea behind this is we get rid of the antibodies in the plasma that contribute to
the immune system's attacks on the peripheral nerves.
This is an idea, a theory, so we think it's how it works, but we aren't 100% certain.
Immunoglobulin Therapy This is when immunoglobulin containing healthy
antibodies, from blood donations is given through the vein.
These treatments are as effective as each other.
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They can be used together, but that does not necessarily make it more effective.
What a person will be treated with depends on their specific case.
Where could you find Guillain-Barré Syndrome in the media?
Guillain-Barre Syndrome was featured in an episode of House MD.
This is specifically episode 15, season 8, Gut Check.
In this episode, a hockey player ends up in the hospital after a fight on the ice.
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He eventually gets diagnosed with a form of Guillain-Barré Syndrome.
If you would like to check out a book, there is Bed Number 10 by Sue Baier and Mary Zimmeth
Schomacker.
This book tells the true story of a woman's 11 month hospital stay after being diagnosed
with Guillain-Barré Syndrome, her experiences with healthcare staff, and the lead up to
her recovery.
If you would like to check out a foundation, there is the GBS CIDP Foundation International.
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They are a global foundation that provides support for those experiencing Guillain-Barré
Syndrome, Chronic Inflammatory Demyelinating Polyneuropathy, and Multifocal Motor Neuropathy.
They provide education to healthcare professionals, caregivers, and those experiencing these conditions
to raise awareness and to better the understanding.
They also fund research into these conditions.
If you want to check out the sources, social media links, or any other links, you can head
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to anatomyofillness.com.
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Did you know the brain is the fattiest organ in the human body, being made up of 60% fat.
These fatty acids are important for many functions.
They also help to provide the structure of the brain.