Let's switch to Myasthenia Gravis.
Many students think of Gravis
as an adult disease.
But there are absolutely, rarely
both in the newborn period
and later on in childhood.
Myasthenia Gravis of the newborn
it presents as a chronic muscle
weakness with fatigability.
This is an autoimmune attack of
antibodies against the Acetylcholine
receptors in the post synaptic
In newborn disease,
this is often because the mother
has Myasthenia Gravis.
So mothers with Myasthenia Gravis
have transplacental migration
of their immunoglobulin or IgG which is
direct against Neuromuscular injuctions.
This crosses the placenta and then attacks
the infants neuro muscular injuction.
Infants may be sick for several weeks
before they resolve as immunoglobulins
can persist for a long time
in the child
after transplacental migration from
Patients present with fatigable weakness
and they may have ocular muscle problems.
This is common especially early
in disease. So they have a disconjugate gaze.
They can also have Ptosis
and they often have diplopia.
Diplopia is not going to be a complaint
that a child is going to have.
But you will see
the disconjugate gaze.
Older children, who are already developing
because they are having their own
will complain of Diplopia.
Myasthenia Gravis is caused by antibodies
directed against the acetyl-choline receptor.
And these antibodies can absolutely be
So the diagnosis is made by simply
checking for the auto antibodies.
However, false negatives can occur.
And in cases where we suspect
we can do nerve conduction
to make that diagnosis more
Another definitive way to make the diagnosis
is to do the Tensilon test.
is effective because
in patients with Myasthenia Gravis,
tensilon will prevent the reuptake of the
acetyl-choline and will cause a transient
increase of Acetyl-choline
in the Neuromuscularinjunction.
As a result, the patient should have
a transient loss of symptoms.
The provision of tensilon should make
the patient essentially improve immediately.
Treatment of Myasthenia Gravis
such as pyridostigmine.
the breakdown of acetyl-choline
breakdown in the neuromuscularjunction
and improve neuromuscular
Additionally, patients can have
to prevent the auto antibodies.
Prednisone is our mainstay
IVIG and plasmaphoresis can
also be used to prevent those
immunoglobulin from attacking
Thymectomy is not recommended
in children the way
it is in adults.
In adults that can be curative.
But in children the thymus is crtically
important for the developing of T-cells.
The thymus is actively involved
in developing those T-cells
So, we will not due thymectomy
on prepubertal children.
That is all I have to talk to you
today about this problem.
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