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Progressive Multicystic Encephalopathy: Is There More Than Hypoxia-Ischemia?

Department of Neonatology, University Charité, Berlin, Germany, lars.garten{at}charite.de

Dieter Hueseman, MD

Department of Neonatology, University Charité, Berlin, Germany

Gisela Stoltenburg-Didinger, MD

Institute of Neuropathology, Unviersity Charité, Berlin, Germany

Ursula Felderhoff-Mueser, MD

Department of Neonatology, University Charité, Berlin, Germany

Katharina Weizsaecker, MD

Department of Obstetrics, University Charité, Berlin, Germany

Ianina Scheer, MD

Department of Pediatric Radiology, Campus Virchow Klinikum, University Charité, Berlin, Germany

Eugen Boltshauser, MD

Department of Pediatrics, University of Zurich, Switzerland

Michael Obladen, MD

Department of Neonatology, University Charité, Berlin, Germany

Progressive multicystic encephalopathy following prenatal or perinatal hypoxia-ischemia is a well-described phenomenon in the literature. The authors report on a term infant with a devastating encephalopathy and severe neuronal dysfunction immediately after delivery without a known antecedent of prenatal or perinatal hypoxia or distress. Clinical and paraclinical findings in the patient are compared with those described in the literature. The authors focus on the specific results guiding to the final diagnosis of progressive multicystic encephalopathy and the timing of morphologic changes. As in this case, if the criteria of an acute hypoxic event sufficient to cause neonatal encephalopathy are not met, then factors other than hypoxia-ischemia may be leading to progressive multicystic encephalopathy.

Key Words: progressive multicystic encephalopathy • MRI

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