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A 23-year-old man was admitted for management of simple partial status epilepticus. He was diagnosed with Parry–Romberg syndrome at the age of 5 years after developing left hemifacial atrophy (figure 1A). Biopsy of a focally hyperpigmented depressed plaque of his left ear showed changes consistent with morphea. He subsequently developed simple partial seizures involving left arm/leg shaking. Serial MRIs revealed progressive right cerebral/cerebellar and left occipital atrophy, all without contrast enhancement (figure 1B). T2 hyperintensities within the right cerebral white matter were noted (figure 1C). Biopsy of an area of such hyperintensity revealed perivascular lymphocytic T cell infiltrates with marked rarefaction and gliosis (figure 1D). There were increased CD3 and CD45 cells, further indicating T cell …
Competing interests None.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.