00:01
After an attack of Guillain-Barre
there's a number of potential
complications that can happen.
00:06
We like to see the early treatment
in with early
initiation of treatment.
00:10
We want to reduce
the nadir of the illness
and see spontaneous recovery
over weeks, months,
or sometimes years.
00:16
Occasionally,
symptoms come back
and there are a couple of
considerations we need to have.
00:20
The first is recurrent AIDP.
00:22
This can happen after
an initial attack of Guillain-Barre,
patients can have
recurrence of symptoms,
but this is rare.
00:30
It occurs in less than 5% of cases.
00:33
It's characterized by
recurrent weakness and numbness,
after the initial attack.
00:37
Classically Guillain-Barre
is a monophasic illness.
00:41
There's rapid onset
early sub-acute onset of symptoms,
it nadirs within 14 days,
and symptoms spontaneously improve
and do not recur.
00:51
Another consideration and a patient
who develops worsening of symptoms
early after their acute attack is
a treatment related fluctuation.
01:00
This occurs as a result of continued
immune destruction of nerves,
which initially improves
with immunomodulatory therapy,
but then continues beyond
the half-life of the treatment.
01:11
So patients are
developing symptoms,
we give them treatment.
01:15
That treatment works temporarily,
but the disease is
continuing to progress.
01:19
And so as the treatment
stops and wears off,
we see the continued
progression of the disease
and this is called a
treatment related fluctuation.
01:26
Patients in this situation
present with recurrent symptoms
several weeks after the initial
treatment of Guillain-Barre.
01:33
They have a nadir, a plateau,
and then ultimately
what we like to see recovery.
01:39
And then lastly,
we may have the diagnosis wrong.
01:42
Patients that have continued
worsening of symptoms.
01:45
More weakness after their
initial presentation
of Guillain-Barre.
01:48
May actually not have
acute inflammatory
demyelinating polyneuropathy,
but chronic inflammatory
demyelinating polyneuropathy.
01:56
A CIDP is a
chronic onset condition,
but occasionally it will present
with a more fulminant
acute presentation.
02:04
The disease does not nadir
at four weeks like Guillain-Barre,
but continues by definition
to persist.
02:10
Symptoms persist
beyond eight weeks.
02:13
This tends to be responsive
to corticosteroids,
unlike Guillain-Barre,
and we do initiate
corticosteroid treatment,
but also use IVIg
and plasmapheresis
to manage these patients.
02:26
Now, let's talk about some of
the variants of typical AIDP.
02:30
There are both axonal variants,
as well as other variants.
02:34
The four that I would like
for you to remember
are acute, motor,
axonal neuropathy,
and that is exactly what it says.
02:41
It's acute and onset
just like Guillain-Barre,
but it's motor predominant.
02:45
There's no sensory symptoms,
and it's an axonal neuropathy.
02:48
We don't see
demyelinating features,
we see axonal features
on our nerve conduction.
02:53
The second is acute motor
and sensory axonal neuropathy.
02:57
So this is very similar.
It's acute and onset,
but there's both
motor and sensory deficits,
and it's also an axonal neuropathy,
and we don't see
demyelinating features.
03:07
The important reason
to call out these axonal variants
of classic Guillain-Barre
is that they have
a worse prognosis.
03:14
Patients may have incomplete
recovery and prolonged weakness
and lack of improvement
back to baseline.
03:21
There are also some other variants.
03:23
There is an autonomic variant
that has prominent autonomic
dysfunction, and dysautonomia,
and then Miller-Fisher syndrome,
which is a unique
bulbar form of this disease.
03:33
Patients present
with ophthalmoplegia,
ataxia, and areflexia.
03:37
And that combination of those
three symptoms that triad
should point squarely to a
diagnosis of Miller-Fisher syndrome.
03:44
This is important to recognize
because prognosis is more favorable.
03:49
And some patients with
Miller-Fisher syndrome
will not even require treatment
not IVIg or plasmapheresis,
with a mild nadir
and gradual or rapid improvement
of symptoms over time.