Prominent microcolumnar cortical architecture in the case of pontocerebellar hypoplasia

• Published in: Folia neuropathologica Vol. 52; no. 4; p. 474
• Main Authors: Laure-Kamionowska, Milena, Szymanska, Krystyna, Lipinska, Elzbieta, Romanska, Justyna, Bekiesinska-Figatowska, Monika, Toborowicz, Jolanta, Raczkowska, Barbara
• Format: Journal Article
• Language: English
• Published: 01.01.2014
• ISSN: 1641-4640; 1509-572X

Pontocerebellar hypoplasia is congenital neuro-developmental disorder characterized by hypoplasia of the cerebellar cortex, dentate nuclei, pontine and olivar nuclei. This report presents a case of 22-week-old infant born at 25 gestational weeks by cesarean section. During hospitalization the neonate underwent sepsis and surgery due to meconium ileus. The infant had seizures treated with phenobarbital and phenytoin. Magnetic resonance imaging (MRI) examination of the head showed posthemorrhagic changes in the cerebellar vermis and cerebellar hemispheres with destruction of cerebellar hemispheres. There is a dilemma whether persistent fetal cerebral cortical circuit is the result of maturational arrest in histogenesis of neocortex or primary cortical dysplasia. It seems that in the presented case the persistent prominent columnar organization of neurons is a result of maturational delay in the cortical development accompanying pontocerebellar hypoplasia.