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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 …
Contributors PD was involved in the drafting and revising the manuscript, conception of the study, analysis and interpretation of data, final approval. GH was involved in revising the manuscript, acquisition of data, analysis and interpretation of data, final approval. EB was involved in revising the manuscript, acquisition of data, analysis and interpretation of data, final approval. MO was involved in revising the manuscript, conception of the study, analysis and interpretation of data, final approval. PD takes responsibility for the integrity of the data and the accuracy of the data analysis.
Competing interests EB has received honoraria for lectures, from Biogen and Bayer Vital. MO has received scientific support, travel support and/or honoraria, from Biogen Idec, Novartis, Sanofi-Aventis, Genzyme, Pfizer, Teva and Heel, as well as receiving research grants from Allergan, Electrocore, Heel and the German Ministry for Education and Research (BMBF).
Patient consent Obtained.
Ethics approval Ethics committee of Asklepios clinics.
Provenance and peer review Not commissioned; externally peer reviewed.