0:01

We've just reviewed a variety of

0:03

cases of different neurocutaneous syndromes

0:05

or phakomatoses.

0:08

Each case is different.

0:09

Each patient is different.

0:12

Some of these entities,

0:13

such as neurofibromatosis type 1

0:15

and tuberous sclerosis complex,

0:17

are more common

0:20

with an incidence of approximately one in

0:21

10,000 individuals, possibly more.

0:25

And these individuals end up getting imaged

0:29

frequently for their variety of lesions.

0:31

They may require treatment, surgical, medical,

0:36

all of which require imaging surveillance.

0:40

Some of these, like neurofibromatosis type 2,

0:43

is more rare,

0:45

potentially 1 in 40,000 individual or less.

0:50

But because of the severity of their lesions,

0:55

it is not uncommon that they get frequent medical

0:59

imaging, so they will be encountered often.

1:01

Others, such as Sturge Weber

1:03

and von Hippel Lindau,

1:04

are not the most common entities,

1:09

also, perhaps one in 40,000 cases.

1:13

But they seek medical care frequently

1:15

for seizures and in the setting of

1:19

Sturge Weber syndrome, and for tumors,

1:22

in the setting of Von Hippel-Lindau.

1:24

Then, there are several other more rare

1:27

neurocutaneous syndromes that we discussed.

1:30

Some of these are associations like

1:32

cloves syndrome and phace association,

1:35

which have only more recently been

1:36

described, and the genetics of which

1:39

have not fully been elucidated.

1:42

All of these conditions are important to be

1:45

familiar with, whether for their amenability

1:49

to multiple-choice questions,

1:50

or more importantly, because the patients that we

1:54

will encounter and hopefully be able to help.