0:00

I have a FLAIR image up of our patient

0:02

with Huntington's Chorea.

0:04

A little bit of quick anatomy.

0:06

The inner and outer aspect of the globus pallidus,

0:09

known as GPI and GPE.

0:14

Then the putamen divided up into two.

0:16

We'll see it a little bit later on.

0:17

There's an inner and outer aspect of the putamen.

0:20

Then the external capsule, then the gray matter,

0:23

claustrom, then the extreme capsule,

0:26

this white stripe,

0:27

and then the centrosylvian cortex.

0:29

I want to briefly take a moment and talk about

0:31

some other metabolic causes of Chorea.

0:35

One of these is hyperthyroidism.

0:37

Now, there's a common theme here.

0:39

Hyperthyroidism drives the mitochondria to produce

0:43

more energy, produce, produce, produce.

0:46

It's basically using its thyroid hormone

0:49

whip against the mitochondria.

0:51

So it's not a coincidence that people with

0:53

Huntington's Chorea develop toxicity at the

0:57

mitochondrial level.

0:58

This is also true for people that have ballism,

1:01

which is a subset of Chorea.

1:04

So this can happen as a manifestation

1:06

of thyrotoxicosis.

1:08

This can also happen as a manifestation

1:10

of hyperglycemia,

1:11

as we discussed in an earlier vignette,

1:14

when you correct the thyrotoxicosis or correct

1:17

the hyperglycemia, the patients get better,

1:19

unless they have prior risk factors for Chorea.

1:23

So, it's very important to know what the patient's

1:26

past medical history consists of.

1:28

There's one other entity that may be associated

1:30

with chorea and also dystonia,

1:33

and that's Wilson's disease.

1:34

It is not a coincidence that this

1:36

is an abnormality of copper.

1:38

And where do you need copper?

1:39

For the oxidative phosphorylative pathway.

1:42

And where is that utilized?

1:45

In the mitochondria.

1:46

So there is a common theme here.

1:48

Mitochondrial toxicity, mitochondrial destruction,

1:51

mitochondrial poisoning in these

1:53

metabolic syndromes.

1:55

Not unlike what occurs in the

1:57

CAG, cytosine adenine guanine repeats

2:01

that produce Huntington's Chorea.