Parry-Romberg syndrome with intracranial manifestations
History of localization-related epilepsy and prior surgical intervention for facial asymmetry. Now presents for imaging evaluation.
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- 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
2 case question available
The parenchymal enhancement seen in the presented case has not been described in the setting of Parry Romberg syndrome; however, the supra and infratentorial white matter signal abnormalities and microhemorrhages or calcifications are in keeping with previously reported findings.
Our patient underwent a brain biopsy which yielded non-specific findings of a lymphoplasmacytic infiltrate. The enhancement and parenchymal signal changes have remained stable over a 3 year follow-up period.
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