Naslov
New neuropathological findings in holoprosencephaly

Autori
Sedmak, Goran ; Mladinov, Mihovil ; Krušlin, Božo ; Judaš, Miloš

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
6th Forum of European Neuroscience ABSTRACT BOOK / - , 2008

Skup
6th FENS Forum

Mjesto i datum
Ženeva, Švicarska, 12.07.2008. - 16.07.2008

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
holoprosencephaly; brain disorder; neurophatology

Sažetak
We describe novel type of neuropathological findings in alobar holoprosencephaly (HPE). A male newborn was born at 30 postconceptional weeks (birth length = 40 cm ; birth weight = 850 g) and died few hours after delivery, with diagnosis of Edwards syndrome, hydramnios and HPE. The brain was fixed in 4% buffered paraformaldehyde, and tissue sections were freezed (for histochemical and immunocytochemical analysis), embedded in celloidin (for Nissl staining) or used for Golgi-Scheibel silver impregnation. Macroscopical features were typical for alobar HPE (agyric/pachygyric holosphere ; the absence of corpus callosum ; monoventricle with obliterated third ventricle, completely fused thalami and partially fused basal ganglia). Nissl staining revealed a typical four-layered cortical plate as well as several novel findings. Two bands of heterotopic neurons were present outside the cortical plate: (a) the first band consisted of numerous cell islands arranged in a single row and situated along the border of the transient fetal intermediate (IZ) and subplate (SP) zones ; that band at low magnification resembled to doublecortex formation (i. e., subcortical band heterotopia) described recently in some cases of X-linked lissencephaly ; (b) the second band of islands of heterotopic neurons was situated within the upper subplate zone, at the border between the subplate and the cortical plate. Both bands of heterotopic neurons consisted of variable proportions of dark and pale cells which probably represented interneurons and projection neurons, respectively. We suggest that the first heterotopic band (at the SP/IZ border) represents a mixture of displaced neocortical and basal ganglia neurons, while the second heterotopic band (at the SP/CP border) represents displaced neurons destined for superficial (cortico-cortically projecting) neocortical layers. Supported by Croatian Ministry of Science, Education & Sport grants No. 108-1081870-1878 (to M. J.) and 108-1081870-1884 (to B. K.).

Izvorni jezik
Engleski

Znanstvena područja
Temeljne medicinske znanosti

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