Incontinentia pigmenti

Case contributed by Dr Sinéad Culleton


15-year-old girl with a known incontinentia pigmenti diagnosis, presented with seizures.

Patient Data

Age: 15 years
Gender: Female

MRI Brain (selected series)

Marked periventricular white matter signal abnormality with atrophy of the superior frontal lobes.

Case Discussion

As incontinentia pigmenti is an X-linked disorder it is more frequently seen in females.

MRI findings include periventricular and subcortical white matter changes, as well as corpus callosum hypoplasia, cerebral atrophy, and cerebellar hypoplasia. This case illustrates periventricular white matter changes.

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Case information

rID: 41192
Published: 20th Nov 2015
Last edited: 14th Aug 2019
Inclusion in quiz mode: Excluded

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