Late progression of neurological symptoms and MRI T2 hyperintensities in Parry-Romberg syndrome

We describe a case of a 50-year-old woman who developed drug-resistant status epilepticus with complex partial and secondary generalised seizures. She had suffered from Parry-Romberg syndrome (PRS) for more than 40 years. Right-sided progressive hemifacial atrophy (PHA) had begun at the age of 7 (figure 1A), followed by epilepsy at the age of 14 years. In the past 2–3 years before the latest admission, the patient had developed a progressive left-sided hemiparesis concomitant with an increase of T2-hyperintensities in the white matter of the right hemisphere, ipsilateral to the PHA (B–D). Moreover, MRI scans illustrated ipsilateral cerebral atrophy (B–D). Blood-sensitive axial imaging revealed evidence of microhaemorrhages or microcalcifications in the right hemisphere (E). Cerebral atrophy was accompanied by contralateral cerebellar atrophy (F). Intrathecal IgG-synthesis as well …