caa a22 4500 46318047X CHVBK 20180406164837.0 cr unu---uuuuu 170326e20070401xx s 000 0 eng 10.1007/s00381-006-0233-1 doi (NATIONALLICENCE)springer-10.1007/s00381-006-0233-1 Midline "brain in brain”: an unusual variant of holoprosencephaly with anterior prosomeric cortical dysplasia [Elektronische Daten] [E. Widjaja, L. Massimi, S. Blaser, C. Di Rocco, C. Raybaud] Introduction: We report three cases of brain malformation presenting with a midline mass of dysplastic cortex that we have termed "brain in brain” malformation. Results: The three cases have holoprosencephalic features, including bilateral hemispheric continuity across the midline, single ventricle, midline facial defect and missing olfactory bulbs. All three cases have a midline conglomerate mass of deeply infolded, cortex-lined fissures with major arterial branches, heterotopia and large amount of white matter. The dysplastic mass of cortex and white matter extended into the third ventricle. The cortex and white matter of the dysplastic lesion was continuous with the cortex and white matter, respectively, of the cerebral hemispheres. Conclusion: The midline "brain in brain” malformations have some similarities to subcortical heterotopia and extracerebral glioneuronal heterotopia. However, the continuity with the cerebral hemispheres and extension into the ventricle were not reported in subcortical or glioneuronal heterotopia. The common involvement of the midline cortex and extension into the third ventricle implied an anterior segmental prosencephalic abnormality (prosomeres 5/6). However, its pathogenesis remains to be explained. Springer-Verlag, 2006 Holoprosencephaly nationallicence Segmental cortical malformation nationallicence Prosomeric malformation nationallicence Subcortical heterotopia nationallicence MR imaging nationallicence Widjaja E. Division of Neuroradiology, Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada aut Massimi L. Pediatric Neurosurgery, Instituto di Neurochirurgia, Università Cattolica del Sacro Cuore, Largo A. Gemelli, 8, 00168, Roma, Italy aut Blaser S. Division of Neuroradiology, Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada aut Di Rocco C. Pediatric Neurosurgery, Instituto di Neurochirurgia, Università Cattolica del Sacro Cuore, Largo A. Gemelli, 8, 00168, Roma, Italy aut Raybaud C. Division of Neuroradiology, Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada aut Child's Nervous System Springer-Verlag 23/4(2007-04-01), 437-442 0256-7040 23:4<437 2007 23 381 https://doi.org/10.1007/s00381-006-0233-1 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00381-006-0233-1 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Widjaja E. Division of Neuroradiology, Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada aut NATIONALLICENCE 700 1- Massimi L. Pediatric Neurosurgery, Instituto di Neurochirurgia, Università Cattolica del Sacro Cuore, Largo A. Gemelli, 8, 00168, Roma, Italy aut NATIONALLICENCE 700 1- Blaser S. Division of Neuroradiology, Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada aut NATIONALLICENCE 700 1- Di Rocco C. Pediatric Neurosurgery, Instituto di Neurochirurgia, Università Cattolica del Sacro Cuore, Largo A. Gemelli, 8, 00168, Roma, Italy aut NATIONALLICENCE 700 1- Raybaud C. Division of Neuroradiology, Hospital for Sick Children, M5G 1X8, Toronto, Ontario, Canada aut NATIONALLICENCE 773 0- Child's Nervous System Springer-Verlag 23/4(2007-04-01), 437-442 0256-7040 23:4<437 2007 23 381 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer