pathology of new variant CJD

Last reviewed 01/2018

Like classical CJD histological examination of brain biopsy and post-mortem specimens of patients with new variant CJD have revealed the presence of spongiform vacuolation, neuronal loss and astrocytosis. These changes are most evident in the basal ganglia and thalmus, with also focal involvement of the cerebrum and cerbellum.

Unlike classical CJD however when plaques occur in some 5% of cases, the presence of kuru-type plaques was consistent amongst the cases of New variant CJD examined.

Immunocytochemistry for prion protein PrP, shows strong staining of the plaques but also reveals the presence of PrP inthe form of small plaques around small neurons in the cerebrum and cerebellum, in areas of the brain where spongiform change was not present.

All definite vCJD cases have occurred in methionine homozygotes at codon 129 in the prion protein gene

Reference:

• Ironside J.W. Variant Creutzfeldt-Jakob disease: an update. Folia Neuropathol. 2012;50:50-56.
• Will RG et al. A New Variant of Creutzfeldt-Jakob disease in the UK. Will RG. et al. Lancet 1996; 347:921-25