Parry-Romberg syndrome with intracranial manifestations

Case contributed by Dr Amin F. Saad


History of localization-related epilepsy and prior surgical intervention for facial asymmetry. Now presents for imaging evaluation.

Patient Data

Age: 45 years
Gender: Female
  • 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.
  • GRE: 
    • 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


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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