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Abstract

We present a novel subtype of neuronal migration disorder found in a case of Pena-Shokeir phenotype, in which the deformation sequence originated from neurogenic fetal akinesia. The autopsied brain showed dysplastic features of dentate and olivary nuclei as well as a bilaterally enlarged olfactory bulb with abnormal laminar structures. The laminar structures comprised four layers: (1) a superficial layer without glomeruli, (2) a neuronal cell layer containing neurons simulating mitral cells, (3) a sparse cell layer enriched with tangential neuronal fibers, and (4) a reticular layer showing a mottled appearance with the accumulation of fine reticular neurites intermingled with granule cells. To our knowledge, these malformed laminar changes have never been reported, and we propose the term olfactory bulb dysplasia.