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With what disease entities does Parry Romberg syndrome share imaging and clinical manifestations?
The extracranial manifestations of PRS overlap with en coup de sabre, a variant of linear scleroderma. The intracranial findings are often nonspecific but do share similarities with Rasmussen encephalitis in that they are typically unilateral.
What are some of the intracranial imaging abnormalities reported in Parry Romberg syndrome?
Intracranial imaging findings include white matter signal abnormality, parenchymal atrophy, cerebral microhemorrhages, parenchymal calcification, and intracranial aneurysms.
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FLAIR:
- pronounced atrophic changes of the left face, with skin thinning, loss of subcutaneous fat, and decreased volume of the muscles of mastication and parotid gland
- abnormal hyperintense signal within the left cerebellar white matter in addition to atrophic changes involving the left masticator space and overlying skin and subcutaneous fat
- confluent signal abnormality within the left centrum semiovale extending to subcortical white matter.
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GRE:
- foci of hypointense signal associated with the left cerebellar white matter FLAIR abnormality, compatible with microhemorrhages or mineralization
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Post-contrast T1 SPGR:
- nodular enhancement within the left cerebellar white matter corresponding to the region of abnormal FLAIR and GRE signal
- multiple foci of nodular enhancement within the region of abnormal FLAIR signal involving the left centrum semiovale and subcortical white matter