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Late progression of neurological symptoms and MRI T2 hyperintensities in Parry-Romberg syndrome
  1. Payam Dibaj1,2,
  2. Gregor Herrendorf1,
  3. Erik Bahn3,
  4. Mark Obermann1
  1. 1Center for Neurology, Asklepios Hospitals Schildautal, Seesen, Germany
  2. 2Max-Planck-Institute for Experimental Medicine, Göttingen, Germany
  3. 3Department of Neuropathology, University of Göttingen, Göttingen, Germany
  1. Correspondence to Dr Payam Dibaj, Center for Neurology, Asklepios Hospitals Schildautal, Karl-Herold-Str 1, Seesen 38723, Germany; p.dibaj{at}asklepios.com

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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 …

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