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This is a CT scan of a nine-year-old child

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with seizures and a right-sided facial

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birthmark called the port wine stain.

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And this patient has Sturge-Weber syndrome.

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You can see there's asymmetric prominence

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to CSF and sulci overlying the right

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cerebral hemisphere compared to the left,

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related to right-sided volume loss.

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We see some focal areas of

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dystrophic mineralization.

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T2-weighted imaging shows, again,

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the prominence of CSF overlying the right

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cerebral hemisphere, related to the

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asymmetric right-sided volume loss.

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We're also seeing a slightly hypointense

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appearance of the juxtacortical white matter

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subjacent to this area.

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And we're seeing extensive leptomeningeal

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enhancement overlying this whole region.

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So, while there's only a very focal area

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of calcification, almost this entire

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area of volume loss has post-contrast

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enhancement on the leptomeningeal surface.

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So, this child was nine years old.

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If we compare their MR at nine years of age

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until their MR at 17 years of age,

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we can see there's been profound progression of

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volume loss in the right cerebral hemisphere.

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There's been some degree of volume loss in the

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left cerebral hemisphere, which is nonspecific.

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It can be related to chronic seizures,

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other issues, but there's definitely

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been a profound progression of the volume

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loss in the right cerebral hemisphere.

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We see this development of asymmetric

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prominence of the diploid space

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overlying the right cerebral hemisphere,

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which is the Dyke-Davidoff-Masson phenomenon

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related to chronic volume loss.

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We see x vacuole enlargement of the atrium and

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occipital horn of the right lateral ventricle as

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compared to the prior study, whereas a similar

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caliber of the atrium and occipital horn of left

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lateral ventricle compared to the prior study.

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Thank you for listening.

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So, this shows the volume loss associated

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with Sturge-Weber syndrome occurs over time.

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It is not a single event.

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This is felt to be related to chronic

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venous congestion, chronic injury,

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and slow progressive volume loss that results in

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dystrophic mineralization, and that volume loss

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due to cell injury or the result in dystrophic mineralization,

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tends to be epileptogenic,

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and therefore, these patients get seizures

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as their brain gets more injured,

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it becomes potentially more epileptogenic.

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So, unfortunately, it has the potential of

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being a feedback loop where the seizures

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eventually get harder and harder to control.

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So, you can see all the areas where there was

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enhancement at nine years of age,

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several years later, had had more profound volume loss.