00:01
So let's talk
a little bit more about
the rapidly progressive ascending
neuropathies or ascending paralysis.
00:07
The typical presentation
is several days
of subacute progressive neuropathy.
00:12
There's prominent weakness.
00:13
Patients may have foot drop,
weakness, difficulty walking,
there's often some
sensory complaints:
numbness, tingling,
burning paraesthesia's
but they're not near as prominent
as the weakness
or motor dysfunction.
00:26
And some other notable symptoms
are back pain is not uncommon.
00:30
And that should not point
towards a spinal cord disorder
in a patient presenting
with this pathology.
00:37
There are some key
clinical questions that we ask
when evaluating patients presenting
with acute rapidly progressive
ascending paralysis.
00:45
One is, is this neuropathy?
Is that a nerve problem or a spinal
cord problem or something else?
The second is
if it's a nerve problem?
Is it inflammatory?
And the third is, is it
demyelinating or axonal?
So let's walk through
each of those steps
in the evaluation
of these patients.
01:01
First, let's talk about
signs and symptoms
of Guillain-Barré Syndrome
at presentation and diagnosis.
01:07
Patients typically present
with symmetrical weakness
starting in the legs.
01:11
At diagnosis this is
present to 90% of patients
10% also starting to involve.
weakness in the arms and face.
01:21
There's a need for
ventilatory support
and 10 to 30% of patients,
face and oropharyngeal weakness
can be seen in 50%.
01:29
Ocular motor weakness
in 15% of patients.
01:32
Areflexia is extremely common.
01:34
90 to 100% of patients
will develop areflexia
and that should point squarely
to a peripheral nerve disorder
as opposed to another localization.
01:43
Pain is not uncommon
in two thirds of patients,
but it's usually not prominent,
and dysautonomia is
extremely common.
01:49
One of the things we need to
watch out for in these patients.
01:53
So these are some of the typical
signs and symptoms that we see
in patients presenting
with Guillain-Barre.
01:58
Now, let's talk about
the typical description of
acute inflammatory
demyelinating polyneuropathy.
02:04
or Guillain-Barre syndrome.
02:06
There are a few features
that I want you to hear
and that we evaluate in patients.
02:10
First is that this
appears acute and onset.
02:14
Now oftentimes, it's not acute
within hours, or minutes, or a day,
but more subacute and onset,
but it's really rapid,
and it has that flavor of being
a rapidly progressive problem.
02:25
The typical onset of symptoms
is subacute
over one to three days.
02:30
It is rapidly progressive.
02:32
The typical Nadir is around
three to four weeks,
and then it plateaus.
02:36
This is a polyneuropathy.
02:38
So we see symptoms
that affect all of the nerves,
both arms and legs,
often symmetrically,
and it can be postinfectious,
after it classically after a
Campylobacter jejuni infection,
but other infections or vaccinations
can lead to this problem.
02:53
And here we're looking at a graph
of some of the things that can occur
classically in patients
with AIDP or Guillian-Barre.
03:00
The first is this
antecedent infection.
03:03
You can see when that occurs,
often a couple of weeks before
the onset of symptoms,
the patients begin
with no weakness.
03:09
And really quickly over time.
03:11
rapidly and progressively
patients develop severe weakness,
many ultimately with paralysis.
03:18
Over time, there's development
of anti-neuronal,
anti ganglioside antibodies,
and it's thought
that this infection
may set off an immune response
and the develop of
these anti neuronal,
anti ganglioside antibodies
that is contributing
to the immune attack.
03:33
The attack nadirs
within about four weeks
and classically within four weeks,
and then patients develop
spontaneous recovery
over weeks to months,
and sometimes even years.
03:46
There are a few other features that
we want to think about with AIDP.
03:50
The second is that it is
an inflammatory disorder.
03:53
This is an autoimmune or immune
mediated attack on the nerves.
03:57
This results in the
ascending weakness
as well as
the early diffuse areflexia
which indicates an immune
mediated proximal polyneuropathy,
which we call a
polyradicular neuropathy.
04:08
This disorder reaches its nadir
of symptoms at 14 days after onset
and can progress
to requiring intubation
secondary to respiratory depression
from respiratory muscle weakness.
04:24
So if we've proven
that this is a neuropathy,
or we're strongly suspicious
of a neuropathic disorder,
our second question is,
is the neuropathy,
is this polyneuropathy
inflammatory?
And here we turn
to our lumbar puncture.
04:37
Lumbar puncture is one
of the most important tests
for evaluating patients
who are presenting with a rapidly
progressive ascending paralysis,
and we're looking for signs
of an inflammatory LP.
04:48
When we perform
an LP or CSF analysis,
we typically look at blood,
white blood cell count
or cell count,
glucose and protein.
04:56
The classic inflammatory LP
as you can see here is normal
to maybe slightly elevated cells
or a pleocytosis
and significantly elevated protein.
05:06
The goal of the LP is to prove
inflammation and exclude infection.
05:11
Some of the things
on the differential diagnosis
for AIDP or Guillain-Barre,
or West Nile polyradiculitis,
CMV polyradiculitis,
and those conditions would
prevent with an infectious LP.
05:25
Our next step is to evaluate
the part of the nerve
that's involved.
05:28
Classically,
Guillain-Barre affects the myelin.
05:31
But there are some variants
that can affect the axon
and have a poor prognosis.
05:35
And we use the nerve
conduction and EMG studies
to confirm that the problem
is neuropathic
to confirm that the problem
is inflammatory,
and to evaluate the part of
the nerve that's involved.
05:46
So our question on the
nerve conduction study is,
do we see a
demyelinating pattern
with prolonged latency and
reduced conduction velocity?
Or an axonal, pattern where there's
reduced amplitude of the responses?
Our EMG can look at the timing
of this condition.
06:00
Do we see acute changes
and spontaneous activity
or more subacute to chronic
changes that would be a typical
for the typical
acute Guillain-Barre.
06:09
And the goal is to determine
whether this is a
demyelinating or axonal disorder?
In terms of the workup, in addition
to LP and nerve conduction,
we may consider MRI of the spine
and patients who present
with atypical features
where we're worried about
a myelopathy.
06:27
Ultimately, lumbar puncture
is the most important tests
to evaluate these patients
and nerve conduction study an EMG
is often used for confirmation.