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.
- 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.
- foci of hypointense signal associated with the left cerebellar white matter FLAIR abnormality, compatible with microhemorrhages or mineralization
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