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Status Epelipticus, CJD, and Encephalitis

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0:01

Let's summarize the findings in herpes encephalitis,

0:04

as one of the diagnoses that is going to be

0:07

made pretty much exclusively by MRI scanning.

0:11

The CT scan findings are typically normal,

0:14

because the abnormality is relatively subtle.

0:16

Herpes will present with fever in 90 percent of cases.

0:20

You'll see it in the peri-insular region,

0:22

as well as in the medial temporal lobes,

0:25

as well as in the cingulum on either side of

0:28

the corpus callosum in the medial frontal lobes.

0:31

Hemorrhage occurs in about one-third of cases, and

0:33

it's usually a bilateral, although asymmetric, process.

0:37

Here, we see that the medial temporal lobe looks

0:40

pretty good on the left side compared to the

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right side, but in the peri-insular region,

0:46

we see bilateral disease in this patient.

0:50

Another entity that may lead to

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seizures and fever is this entity.

0:57

So here, we have diffusion-weighted imaging

1:01

showing high signal intensity in the cortex.

1:04

And this is a bilateral process.

1:07

It's a little bit asymmetrical.

1:09

In a patient who is having status epilepticus,

1:13

you can see cytotoxic edema of the gray matter,

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which will reverse without permanent damage.

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So, in a patient who is actively seizing in the

1:24

MRI scanner, has recently stopped seizing,

1:28

or is being controlled in status epilepticus,

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this is a pattern that you may see that is

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limited to the cortex as cytotoxic edema on DWI.

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However, the differential diagnosis includes

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those entities that cause encephalitis,

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which include a number of viral etiologies,

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including things like measles, or mumps, or rubella,

1:54

or other infectious etiologies, West Nile virus,

1:58

etc. All of these can cause cortical edema.

2:02

So the differential diagnosis is: does the patient

2:06

have a primary seizure disorder, and what we're

2:09

seeing is cytotoxic edema secondary to recent

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seizures, or does this patient have encephalitis?

2:17

And we're seeing cytotoxic

2:18

edema because of the infection.

2:20

Obviously, this is easier made by the

2:22

clinicians, because if the patient has a

2:24

fever, we're more likely to have encephalitis.

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If the patient has a known seizure disorder that's

2:29

out of control, or if the patient has stopped taking

2:32

their seizure medications, then that might be the

2:34

reason why the patient is in status epilepticus.

2:38

In this case, I fooled you.

2:41

This is a patient who has

2:42

Creutzfeldt-Jakob disease, CJD.

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CJD is a disease entity that is due to an

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infectious prion particle, and it can lead to

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seizures, fever, as well as acute mental status

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changes and a rapidly developing encephalopathy.

3:04

CJD most commonly affects the cortex.

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And second, most commonly affects the deep gray matter.

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However, sparing of the deep gray matter should not

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dissuade you from suggesting the diagnosis of CJD.

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Unfortunately, at this time in 2021, we really

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don't have any good medication for treating CJD.

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It's just expectant therapy with

3:30

anti-seizure medications, etc.

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So this is a patient who has encephalitis, and

3:36

that encephalitis is what we call a neurodegenerative

3:39

disorder of Creutzfeldt-Jakob disease.

Report

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Neuroradiology

MRI

Infectious

Emergency

Brain

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