Pathology

Creutzfeldt jacob disease is characterised by the demonstration at autopsy of the classical histological triad of spongiform vacuolation, astrocytic proliferation and neuronal loss.

The spongiform vacuolation may affect any part of the grey matter and often there is marked neuronal loss. In spite of this latter feature however there is little if any gross atrophy of the brain, presumably because the disease process is so rapid, unlike in more chronic forms of dementia such as Alzheimer's disease where there is marked atrophy.

In some 5% of cases of Creutzfeldt Jakob disease these histological changes are accompanied by the presence of amyloid plaques.

Reference:

• New Diagnostic tests for prion diseases.Collinge J. New England Medical Journal 1996 vol 335(13)963-965.
• A New Variant of Creutzfeldt-Jakob disease in the UK. Will RG. et al. Lancet 1996; 347:921-25