0:00

I'd like to wrap up Chorea by showing

0:02

you what happens in the endgame

0:05

when you have metabolic or toxic-related phenomena

0:09

at the mitochondrial oxidative

0:12

or oxygen producing ATP-producing level,

0:17

you've got necrosis of both putamina,

0:20

especially in metabolic disorders.

0:23

And these are frequently symmetric in both putamina,

0:26

but also potentially in the thalami,

0:29

although the thalami are not involved.

0:31

The posterior putamen is more apt to be involved

0:35

than the anterior putamen.

0:37

Now, in certain metabolic disorders,

0:39

the globus pallidus may be affected.

0:41

One of these would be carbon monoxide poisoning,

0:44

which also affects the oxidative ATP supply.

0:50

Common to these disorders is the movement theme

0:54

of Chorea and the symmetry of involvement.

0:58

But there are certain choreoform disorders

1:00

that are asymmetric.

1:01

You've seen Sydenham's Chorea,

1:03

classically caused by rheumatic fever,

1:06

which is more often asymmetric than symmetric.

1:09

The metabolic ones, though,

1:11

tend to be symmetric like Wilson's disease,

1:13

an abnormality of copper, intimately involved

1:17

in the oxidative process.

1:19

Leigh's disease,

1:20

an abnormality of pyruvate carboxylase

1:23

and related enzymes.

1:25

Vascular insults,

1:27

which can be symmetric or asymmetric due to

1:30

lenticulostriate perforating infarcts

1:34

at the base of the brain.

1:36

Other causes of choreoathetotic bilateral

1:39

putaminal necrosis or thalamic necrosis include

1:44

any other mitochondrial disorder,

1:46

lipid metabolism disorders, vitamin disorders,

1:50

especially vitamin B1

1:52

seen in Wernicke's encephalopathy,

1:55

which produces bilateral putaminal and thalamic

1:59

necrosis along with mammillary body hemorrhage and

2:01

necrosis, certain degenerations like multisystem

2:05

atrophy, immune-mediated disorders like systemic

2:09

lupus erythematosus, and a very important one,

2:13

antiphospholipid antibody syndrome,

2:15

seen in women producing an increased risk of miscarriage

2:19

and then inflammatory disorders like HIV,

2:23

which is the most important common cause of a

2:27

choreaform disorder with putaminal involvement,

2:30

and then not seen so commonly, Rubella.

2:33

Putaminal necrosis,

2:35

where the signal starts to match that of

2:37

cerebrospinal fluid on the T1

2:39

and on the T2 weighted image,

2:41

not unlike symmetric

2:44

thalamic abnormalities,

2:46

with a reasonable laundry list of a differential diagnosis.

2:49

Let's move on, shall we?