Multiple cerebral cavernous malformations

Case contributed by Dr James Harvey

Presentation

Seizures since infancy. Presented to ED following a prolonged seizure.

Patient Data

Age: 20 years
Gender: Female
CT

CT head

Several dense calcified lesions are seen. A well-circumscribed lesion involves the cingulate gyrus and body of the corpus callosum.
Scattered calcification is also seen within the right frontal lobe with surrounding hyopdensity. 

There is no enhancement on the post-contrast study.

MRI

MRI brain

Multiple areas of SWI blooming artefact representing cavernous malformations are seen within the supra and infratentorial brain parenchyma. The largest one in the right frontal region is heterogeneous with predominant hyperintense foci involving the grey and white matter.  Blooming artefacts noted within in keeping with blood products.
The adjacent grey and white matter shows atrophy.  Ipsilateral mild dilatation of the frontal horn of the lateral ventricle is likely related to gliosis.  

Numerous other cavernous malformations are seen.

There is no significant perilesional edema. No mass effect or midline shift.  

Case Discussion

Cavernous malformations are usually isolated. Where multiple cavernous malformations are present, familial multiple cavernous malformation syndrome must be considered. This condition typically presents with seizures. The inheritance pattern is autosomal dominant, with variable penetrance.

This patient had undergone previous surgical evacuation of hemorrhage within the right frontal lobe.

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Case information

rID: 70900
Published: 9th Sep 2019
Last edited: 12th Sep 2019
Inclusion in quiz mode: Included

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