A 19-year-old woman with relevant past medical history of migraines without aura and localized scleroderma involving her left scalp presented to the emergency department following a second Jacksonian seizure in two months. A 1.5T MRI of her brain demonstrated mild left cerebral hemiatrophy and T2 hyperintense lesions located within the left parietal corona radiata, the left dorsolateral thalamus, and the left inferior frontal gyrus. She had a routine EEG that was unremarkable. Having returned to her cognitive and functional baseline she was discharged from the emergency department on an antiepileptic with neurology clinic follow-up scheduled. When she was seen in neurology clinic a few months later she had remained seizure free. A 3T MRI with contrast was done that showed similar findings and subtle enhancement of the left parietal and left frontal lesions. Over the following year the patient continued to remain seizure free, without development of any focal deficits, and her repeat imaging remained vastly unchanged. However, her migraines became more frequent and despite being immunosuppressed with close following by dermatology and rheumatology she developed a new progressive skin lesion over her left forehead.

Progressive Hemifacial Atrophy (PHA), also known as Parry-Romberg syndrome, is a sporadically acquired slowly progressive disease of unknown etiology that often has neurologic involvement. This patient exhibited focal epilepsy and migraines which are two of the more common neurologic manifestations. Another known manifestation is trigeminal neuralgia. It should be known that cerebral vascular malformations and cerebral aneurysms have been associated with PHA and a case of ischemic stroke has been reported. Her white matter T2 hyperintense lesions on MRI are most typical in the characteristic appearance and the involvement of the hemisphere ipsilateral to the skin lesions. Her finding of cerebral hemiatrophy is a rare but known association.