Teaching NeuroImages: Progressive facial hemiatrophy (Parry-Romberg syndrome) with ipsilateral cerebral hemiatrophy

A 52-year-old man presented with left hemifacial at-rophy (figure 1) beginning at age 25. There were no neurologic symptoms. Neurologic examination showed no deficits, and the limbs were symmetric. Brain MRI demonstrated left cerebral hemiatrophy (figure 2) and lack of the ipsilateral soft facial tissue. Progressive facial hemiatrophy (PFH), or Parry-Romberg syndrome, is a sporadic disease of unknown etiology characterized by progressive shrinking and de-formation of one hemiface with subcutaneous connec-tive and fatty tissue atrophy.1,2 Rarely, brainMRI shows cerebral hemiatrophy, usually ipsilateral to the facial at-rophy. PFH can present with neurologic symptoms such as trigeminal neuralgia and focal epilepsy.1,2 Histo-logic findings reveal a proliferative interstitial neurovas-culitis.3 Chronic localized meningoencephalitis with vascular involvement may be a cause of the occasional brain involvement in PFH.3 The coexistence of brain and facial atrophy on the same side suggests that facial atro-phy is not caused by brain injury. Rasmussen encephalitis, however, may also be associated with PFH, suggesting that these 2 conditions may share a common etiology.