0:00

I'd like to summarize what we've learned

0:03

in the sections through the demyelinating

0:06

and dysmyelinating disorders of

0:08

the central nervous system.

0:09

We initially looked at normal white matter

0:12

and then went to the most common of the

0:16

young adult neurodegenerative disorders,

0:18

that being multiple sclerosis.

0:20

Multiple sclerosis is the most common of

0:23

the demyelinating disorders once one

0:25

eliminates the quote-unquote small vessel

0:28

ischemic disease of the elderly.

0:30

We saw different variants

0:32

of multiple sclerosis,

0:33

including some cases in which the patient

0:36

presented with tumefactive

0:38

demyelinating lesions,

0:40

large white matter lesions

0:42

over 2 cm in size,

0:44

and they ultimately went on

0:45

to multiple sclerosis.

0:47

We also learned about clinically isolated

0:50

syndrome and the importance of the MRI

0:53

being performed in those

0:54

patients for prognosis,

0:56

as far as whether the patient will

0:58

progress to multiple sclerosis and the

1:01

related entity is radiologically

1:04

isolated syndrome,

1:05

where one has a patient who does not have

1:07

neurologic symptoms associated with

1:10

multiple sclerosis or that diagnosis.

1:12

And yet the MR.

1:13

Findings are classic in fulfilling the

1:17

McDonald criteria for multiple sclerosis.

1:22

We also looked at some of the differential

1:25

diagnosis for high-intensity

1:26

foci in the young,

1:28

and we used the term age-appropriate

1:32

or greater than expected for age,

1:34

and these may be white matter lesions out

1:37

in the periphery that can occur in

1:38

patients with migraines can occur

1:41

after head trauma. For example,

1:43

we know that there is a definite

1:45

leukocencephalopathy in soccer players

1:48

who head the ball repetitively,

1:52

that they do show abnormalities in their

1:54

white matter, particularly on diffusion,

1:56

tensor imaging, and ADC mapping.

1:59

So those are some of the etiologies

2:01

that might consider.

2:02

With white matter disease in the young,

2:05

that is, spotty.

2:07

We emphasize the clinical history for some

2:09

of the cases of white matter

2:12

demyelinating disorders,

2:14

such as progressive

2:15

multifocal leukoencephalopathy.

2:17

Usually, a patient who is immunosuppressed

2:20

may have AIDS, may be an organ transplant,

2:24

may have medications that

2:26

are immunosuppressive,

2:27

and that would be a key to that diagnosis

2:30

of progressive multifocal

2:32

leukoencephalopathy.

2:34

We also impressed upon the listener the

2:38

importance of the history and the

2:40

laboratory findings in central

2:43

pontine myelinolysis,

2:44

which we recalled as osmotic

2:47

demyelination,

2:48

so fluctuations in the sodium and

2:51

potentially the potassium.

2:53

A similar clinical history may be useful

2:55

for the diagnosis of acute disseminated

2:57

encephalomyelitis, in that the.

3:00

Patient usually has an antecedent viral

3:03

infection or vaccination and therefore

3:06

occurs more commonly in children.

3:09

Finally,

3:09

we talked about various unusual vascular

3:12

diseases above and beyond small vessel

3:15

ischemic disease or microangiopathic

3:17

arteriosclerosis,

3:18

and we looked at patients who have caticil

3:22

and binswangers or SAE and looked at the

3:25

differential diagnosis emphasizing the

3:28

importance of white matter changes in the

3:31

anterior temporal lobe extending to the

3:33

subcortical U fibers with caticil

3:37

as well as the external capsule,

3:40

as opposed to those patients with bin

3:42

swangers or SAE where one has a

3:45

predominance of central white matter

3:47

disease as well as lacuna infarctions

3:50

involving the basal ganglia

3:52

and central structures.

3:54

At the end,

3:55

we talked a little bit about

3:57

demyelinating disorders and the ones

3:59

that I emphasized were metachromatic

4:01

leukodystrophy because in general that is

4:03

the most common and has the stereotypical

4:07

features of sparing of the subcortical

4:10

U fibers and the tigroid stripes.

4:12

And we contrasted that with the two

4:15

entities that are associated with

4:17

megaleencephaly or macrocephaly,

4:19

those being Alexander's disease and

4:22

Canavan's disease. Canavan's disease.

4:25

We emphasize the role of Mr.

4:27

Spectroscopy in showing the marked

4:30

elevation of the NAA.

4:32

We then talked about adrenal

4:34

leukodystrophy,

4:34

which is the one demyelinating disorder that

4:38

favors the posterior white matter and has

4:41

that contrast-enhancing leading edge

4:44

of the myelin breakdown in this

4:48

demyelinating disorder associated

4:50

with hypoadrenalism and

4:52

visual disturbance.

4:55

And then we talked a little bit about some

4:56

of the pearls for the rest of the eponyms

4:59

that may lead to a correct diagnosis.

5:02

So the white matter in the brain,

5:03

the white matter in the bion fascinating

5:06

area of anatomy,

5:07

and it leads to a potpourri of diseases

5:11

that cause demyelinating and

5:13

dismyelinating disorders.

5:15

Thank you very much for your attention.