Parry Romberg syndrome with intracranial manifestations

Case contributed by Dr Amin F. Saad


A 45-year-old female with a history of localization-related epilepsy and prior surgical intervention for facial asymmetry presents for imaging evaluation.

Patient Data

Age: 45 years
Gender: Female

Multiple axial MR images demonstrate pronounced atrophic changes of the left face in addition to abnormal hyperintense FLAIR signal, foci of susceptibility artifact, and nodular enhancement within the left cerebral and cerebellar white matter. 

Case Discussion

This case illustrates the typical extracerebral manifestations of Parry Romberg syndrome with pronounced hemifacial atrophy in addition to less common intracranial findings.

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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Case information

rID: 50949
Case created: 30th Jan 2017
Last edited: 8th Feb 2017
Inclusion in quiz mode: Included

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