0:00

We can categorize or go at neurodegenerative

0:03

diseases from the disease type,

0:05

from the age distribution, from the heredity,

0:08

or from the actual movement disorder.

0:10

I'd like to start out with the movement disorder

0:12

and the one I'd like to begin with is Chorea.

0:15

This entered medical writings through descriptions

0:18

of religiously inspired outbreaks of

0:21

mass hysteria in the Middle Ages.

0:24

There was an association with various saints at

0:27

the time and the worshipers called on the saint, St. Vitus,

0:31

and this became widely known as the St. Vitus dance.

0:35

In fact,

0:37

this dance could be a little bizarre and violent,

0:40

so that individuals were sometimes put to death,

0:43

thought to be witches.

0:44

This persisted as a synonymous term for Chorea

0:47

in 20th-century textbooks of neurology.

0:51

Now, there's an association between

0:54

Chorea and injury or inflammation,

0:57

especially of the putamen of the corpus striatum.

1:00

This is known as Sydenham's Chorea.

1:02

It can be found in rheumatic fever,

1:05

endocarditis, rubella, and other infectious

1:08

and inflammatory processes.

1:10

And this entity of Sydenham's Chorea was

1:12

appreciated before the 19th century.

1:14

Often overlooked

1:16

is Chorea occurring as a result of trauma,

1:20

often from a unilateral bleed into the corpus striatum,

1:24

especially the putamen.

1:26

Eventually though,

1:28

it became clear that there was a hereditary Chorea

1:31

through the concise report by George Huntington

1:34

on affected families in New York.

1:37

Now, we all think everything happens

1:39

and starts in New York,

1:40

but Huntington's Chorea probably

1:42

did not start there.

1:43

It probably started in the countries where the

1:46

incidence of Huntington's Chorea is the highest.

1:49

And those two countries are Scotland and Venezuela.

1:53

Most likely, this disorder inherited

1:56

as an autosomal dominant,

1:58

affected a single individual with a mutation.

2:02

And that mutation propagated amongst the

2:05

family members and progeny.

2:07

And either those individuals emigrated from

2:11

Scotland to Venezuela or from Venezuela to

2:13

Scotland, and then spread to the rest of the world.

2:16

Let's move on, shall we?