Neuroradiology

Dysgenesis, cerebellar

congenital abnormal formation of the cerebellum and manifested in different forms and degrees of severity:

  • total or subtotal agenesis

  • lateral aplasia

  • median aplasia

  • hypoplasia

    Near total aplasia of the cerebellar vermis, dysplasis and heterotopias of cerebellar nuclei, absence of pyramidal decussation, anomalies of the inferior olivary nuclei are found in Jouberts syndrome.

    Rhombencephalosynapsis is characterized by absence of the cerebellar vermis and fusion of the cerebellar hemispheres.

    Lhermitte Duclos disease is a cerebellar dysplasia characterized by thickening of the cerebellar folia due to a cellular disorganization with hypertrophied granula cell neurons and axonal hypermyelination in the molecular layer (dysplastic gangliocytoma).

    GS