0:00

This is a child who presented with shunt

0:04

failure to the emergency room

0:06

with no prior examinations.

0:08

At this juncture for shunt failure,

0:11

we're typically performing MR

0:13

sequences with HASTE fast T2-weighted scans

0:18

in the sagittal axial and coronal plane,

0:20

and we don't necessarily perform CT scan.

0:24

This study, however,

0:25

was performed in 2014 before

0:27

we implemented an MR protocol

0:30

for evaluation for children with hydrocephalus.

0:33

So, let's look at the CT scan.

0:35

What we see with the CT scan is a patient

0:38

who has bilateral ventriculostomy catheters

0:41

with bilateral areas of calcification in the

0:47

basal ganglia region,

0:48

as well as in the periphery.

0:52

You see some calcification

0:54

in the periphery here,

0:55

and the patient has cysts within the white

0:59

matter in the parenchyma of the brain.

1:03

Now, on initial blush,

1:05

we might consider torch infections,

1:08

which may lead to the calcifications in

1:11

the periventricular region.

1:13

However, this wouldn't necessarily account for some

1:15

of the more peripheral calcifications.

1:18

The combination of the calcifications

1:21

associated with parenchymal cysts

1:24

might lead us to worry about a

1:27

dysmyelinating disorder that would also have

1:30

associated calcifications and cysts.

1:33

And the dysmyelination might be brought up

1:36

by the fact that there is low density

1:38

within the white matter

1:40

bilaterally in this patient.

1:42

So, we'd be considering a leukodystrophy with

1:46

calcification and cysts

1:47

versus a torch infection.

1:49

This patient subsequently

1:51

went on to MRI scanning.

1:53

Looking at the FLAIR scan,

1:56

we see the large parenchymal cysts,

2:00

which has signal intensity,

2:02

which is not the same as the

2:04

CSF of the ventricle,

2:05

and we see that there actually are multiple

2:08

ones of these various cysts.

2:11

In addition,

2:12

we have the white matter disease that is

2:14

predominantly in the temporal

2:15

lobe and frontal lobe,

2:19

and we see the residual from the

2:22

ventriculostomy catheterization.

2:27

The calcifications are seen in the basal

2:29

ganglia, the thalamus, etc.

2:33

And this patient has a diagnosis of

2:37

leukoencephalopathy with cysts

2:40

and calcification.

2:41

It is one of the more obscure of the

2:44

leukodystrophies that can occur,

2:47

and the white matter disease may be diffuse

2:49

or it may be more selective to the temporal

2:52

lobes or even in the cerebellum.

2:54

Here you see that there is involvement of

2:56

the pons, but not so much involved

3:00

and of the central cerebellum.

3:02

This used to be called Labrune syndrome,

3:05

but we now refer to it as leukoencephalopathy,

3:09

with parenchymal cysts and calcifications.