Our topic now brings us to
progressive supranuclear palsy.
Progressive supranuclear palsy
characterized by truncal rigidity
There will be nuchal dystonia.
What does dystonia mean?
Contraction of muscle that’s taking
place for long period of time.
Pseudobulbar palsy and abnormal
speech, ocular disturbance,
and here, it’s vertical gaze palsy,
which is extremely important,
and mild progressive dementia.
The clinical pearl here is going
to be vertical gaze palsy.
Progressive supranuclear palsy.
Its onset is between the fifth
and seventh decades of life
and studies have shown that males and
females could share equal preponderance.
So what’s happening in
progressive supranuclear palsy?
Neuronal loss in the globus pallidus.
Think about that for one second.
substantia nigra, colliculi,
periaqueductal gray matter and
dentate nucleus of the cerebellum.
There’s neural loss in many of these
structures that we’ve identified here.
could also be found,
but remember neurofibrillary tangles
is something that we find in
every individual who gets
older, elderly, okay?
Those neurofibrillary tangles are
structurally similar to what
is seen in Alzheimer’s, but the chemical
composition is different.
However, if the patient starts showing
you signs of Alzheimer’s disease,
then the number of neurofibrillary
tangle is going to then correlate
with the severity of
the disease itself.
Our topic here is corticobasal
type of degeneration.
It’s a disease of the elderly
characterized by extrapyramidal rigidity.
Asymmetric motor disturbances.
In other words, a multifocal type of
myoclonus and sensory cortical dysfunction.
By cortical dysfunction, you
should be thinking about
higher order type of activity including
apraxia, disorder of language,
and then here, alien
hand type of phenomenon.
All of this is referred to as being your
corticobasal type of degeneration.
Allow the name to speak to you.
The pathology here will
be cortical atrophy
of motor, premotor, or perhaps
even anterior parietal lobes.
Hence, we talked about the language
disorder that might be taking place,
and we talked about that alien
hand type of phenomenon.
Neuronal achromasia: severe loss of
neuron, gliosis, and ballooned neurons.
Corticobasal, ballooned neuron.
Substantia nigra and locus ceruleus
show loss of pigmented neurons
and something that we refer to as being
your argyrophilic type of inclusions.
Corticobasal type of degeneration.
type of atrophy.
It’s a spectrum of diseases, characterized
by presence of glial cytoplasmic inclusion,
typically with cytoplasm
So here, when you think about
multiple system atrophy,
it’s a spectrum, and we’ll talk about
this a little bit moving forward.
Well, here, we have something called
striatonigral type of degeneration.
This as you can expect very much so will
be movement disorder resistant to L-Dopa
because literally, it’s a destruction or
degeneration of the nigrostriatal pathway.
Atrophy of the caudate nucleus
and putamen is involved.
Nuclei show extensive neuronal
loss and marked gliosis.
Remember, gliosis always means your repair
mechanism for the brain, doesn’t it?
And when we say repair
mechanism in the brain,
remember that we do not have
fibroblasts in the brain.
So therefore, there is no
scar formation technically.
Yet, we call it a glial scar and we
talked about this in general pathology
that that “glial
scar” is a misnomer
and the fact that we do have microglia
that are coming in to remove the debris,
but gliosis to you should
mean rather marked damage
or that injury is taking
place to the brain itself.
Under multiple system atrophy,
we have a couple other variants
that I’ll quickly
walk you through.
Let’s go with
It’s an extrapyramidal syndrome that
combines your autonomic system dysfunction.
So by this we mean that if you’re
lying in a supine position
where at this point you have increased
blood flow to the heart, supine position,
and then all of a sudden you
go from a supine or even
a sitting position to a
standing up position,
then you’re going to pool your blood
down in the legs very quickly.
And if you have autonomic
type of instability,
then please understand that you’re
going to exhibit what’s known as
Impotence, your sweat
glands and, in general,
your glands are not working properly
and papillary abnormalities.
Another type of multiple system atrophy,
with something called
As the name implies,
there will be cerebellar
degeneration that’s taking place.
And as soon as the cerebellum
is being affected,
then you can expect there
to be a type of ataxia.
Eye and somatic
and then rigidity as well.