Fig. 2.

Immunohistochemical findings in the cerebellum of the second affected foetus. a GFAP immunolabeling of the cerebellar white matter showing immature astrocytes possessing coarse or thin and short cell processes [OM × 400]. b Contrary to the control cerebellar white matter which contains numerous mature astrocytes with multiple fine processes [OM × 400]. c Nestin immunostaining demonstrating overexpression of this intermediate filament in immature astrocytic cell somata [OM × 400]. d Contrary to control cerebellar white matter in which nestin expression is mainly restricted to some astrocytic processes [OM × 400]. e Intense PDGFRα immunoreactivity of the oligodendrocyte precursors in the patient’s cerebellar white matter [OM × 400]. f Contrasting with almost absent PDGFRα expression in the control cerebellar white matter [OM × 400]. g Virtually absent GFAP-immunoreactivity of the Bergmann glia in the patient’s cerebellar cortex [OM × 200]. h Compared with the control cerebellar cortex in which Bergmann cells are located adjacent to Purkinje neurons (arrow), observed throughout the molecular layer up to the transient external granular cell layer [OM × 200]. i Patient’s cerebellar cortex exhibiting weak calbindin immunoreactivity in the small immature and dysmorphic Purkinje cells devoid of apical dendritic arborizations (asterisk), forming a discontinuous layer [OM × 100]. j Whereas in control cerebellum, Purkinje cell somata and dendritic trees (asterisk) are strongly calbindin-immunoreactive as are stellate cells (thin arrow) and Golgi II neurons (thick arrow) [OM × 100]. OM: original magnification