caa a22 4500 445376341 CHVBK 20180317143003.0 cr unu---uuuuu 170323e20110901xx s 000 0 eng 10.1007/s00702-011-0609-6 doi (NATIONALLICENCE)springer-10.1007/s00702-011-0609-6 Rare brain biopsy findings in a first ADEM-like event of pediatric MS: histopathologic, neuroradiologic and clinical features [Elektronische Daten] [Franziska Hoche, Sabine Pfeifenbring, Stefan Vlaho, Mayyada Qirshi, Marius Theis, Wilfried Schneider, Luciana Porto, Klaus Müller, Matthias Kieslich] Pediatric MS tends to present more often with an acute onset and a polysymptomatic form of the disease, possibly with encephalopathy and large tumefactive lesions similar to those observed in some cases of acute disseminated encephalomyelitis (ADEM), which makes it more difficult to differentiate between an explosive and severe onset of MS vs. ADEM. An ADEM-like first demyelinating event can be the first attack of pediatric MS, but international consensus definitions require two or more non-ADEM demyelinating events for diagnosis of MS. In our patient KIDMUS MRI criteria for MS (Mikaeloff et al. J Pediatr 144:246-252, 2004a; Mikaeloff et al. Brain 127:1942-1947, 2004b) were negative at first attack, but Barkhof criteria for lesion dissemination in space in adults (Barkhof et al. 120:2059-2069, 1997), Callen modified MS-criteria and Callen MS-ADEM criteria for children (Callen et al. Neurology 72:961-967, 2009a; Callen et al. Neurology 72:968-973, 2009b) were positive suggesting pediatric MS. As the clinical course was devastating with non-responsiveness upon high-dose immune modulatory therapy and due to the absence of an alternative diagnosis other than demyelinating disease brain biopsy was performed. Brain biopsy studies or autopsy case reports of fulminant pediatric MS patients are extremely rare. Histopathology revealed an inflammatory demyelinating CNS process with confluent demyelination, indicating the likelihood of a relapsing disease course compatible with an acute to subacute demyelinating inflammatory disease. This pattern was corresponding to the early active multiple sclerosis subtype I of Lucchinetti et al. (Ann Neurol 47(6):707-717, 2000). Springer-Verlag, 2011 Pediatric multiple sclerosis nationallicence ADEM nationallicence MRI diagnostic criteria nationallicence Plasma exchange nationallicence International MS Study Group nationallicence MS histopathology nationallicence Hoche Franziska Department of Neuropediatrics, Goethe University Frankfurt, Theodor-Stern-Kai 7, 60590, Frankfurt, Germany aut Pfeifenbring Sabine Department of Neuropathology, University Medical Center Göttingen, Robert-Koch Str. 40, 37099, Göttingen, Germany aut Vlaho Stefan Department of Neuropediatrics, Goethe University Frankfurt, Theodor-Stern-Kai 7, 60590, Frankfurt, Germany aut Qirshi Mayyada Department of Neuropediatrics, Goethe University Frankfurt, Theodor-Stern-Kai 7, 60590, Frankfurt, Germany aut Theis Marius Department of Neuropediatrics, Goethe University Frankfurt, Theodor-Stern-Kai 7, 60590, Frankfurt, Germany aut Schneider Wilfried Department of Pediatric Intensive Care Medicine, Goethe University Frankfurt, Frankfurt, Germany aut Porto Luciana Department of Neuroradiology, Goethe University Frankfurt, Frankfurt, Germany aut Müller Klaus Department of Neurology, Edinger Institute, Goethe University Frankfurt, Frankfurt, Germany aut Kieslich Matthias Department of Neuropediatrics, Goethe University Frankfurt, Theodor-Stern-Kai 7, 60590, Frankfurt, Germany aut Journal of Neural Transmission Springer Vienna 118/9(2011-09-01), 1311-1317 0300-9564 118:9<1311 2011 118 702 https://doi.org/10.1007/s00702-011-0609-6 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00702-011-0609-6 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Hoche Franziska Department of Neuropediatrics, Goethe University Frankfurt, Theodor-Stern-Kai 7, 60590, Frankfurt, Germany aut NATIONALLICENCE 700 1- Pfeifenbring Sabine Department of Neuropathology, University Medical Center Göttingen, Robert-Koch Str. 40, 37099, Göttingen, Germany aut NATIONALLICENCE 700 1- Vlaho Stefan Department of Neuropediatrics, Goethe University Frankfurt, Theodor-Stern-Kai 7, 60590, Frankfurt, Germany aut NATIONALLICENCE 700 1- Qirshi Mayyada Department of Neuropediatrics, Goethe University Frankfurt, Theodor-Stern-Kai 7, 60590, Frankfurt, Germany aut NATIONALLICENCE 700 1- Theis Marius Department of Neuropediatrics, Goethe University Frankfurt, Theodor-Stern-Kai 7, 60590, Frankfurt, Germany aut NATIONALLICENCE 700 1- Schneider Wilfried Department of Pediatric Intensive Care Medicine, Goethe University Frankfurt, Frankfurt, Germany aut NATIONALLICENCE 700 1- Porto Luciana Department of Neuroradiology, Goethe University Frankfurt, Frankfurt, Germany aut NATIONALLICENCE 700 1- Müller Klaus Department of Neurology, Edinger Institute, Goethe University Frankfurt, Frankfurt, Germany aut NATIONALLICENCE 700 1- Kieslich Matthias Department of Neuropediatrics, Goethe University Frankfurt, Theodor-Stern-Kai 7, 60590, Frankfurt, Germany aut NATIONALLICENCE 773 0- Journal of Neural Transmission Springer Vienna 118/9(2011-09-01), 1311-1317 0300-9564 118:9<1311 2011 118 702 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer