Table 3.

Phenotypical CJD characteristics

	Molecular subtype	Clinical features	Neuropathology	PrP immunohistochemistry
Frequent	MM1/MV1	dementia, cortical anopsy, myoclonia, short duration of disease (approximately 4 months)	pronounced damage of the occipital cortex, PrP accumulations of the synaptic type
	MV2	ataxia, dementia, extrapyramidal motion disorder, long duration of disease (approximately 18 months)	focal damage of the cortex, amyloid (kuru) plaques, focal plaque-like PrP accumulations
	VV2	ataxia at onset, dementia at a late stage, average duration of disease (approximately 7 months)	pronounced damage of the subcortical structures as well as the brainstem, often spongiosis only located in the deeper cortical layers, plaque-like as well as perineuronal PrP accumulations
Rare	MM2 thalamic (sFI)	insomnia, dysautonomia, at a late stage ataxia and cognitive disturbance	atrophy of the thalamus and the nucleus olivaris, lack of spongiosis possible
	MM2 cortical	dementia for several months	large confluent vacuoles with perivacuolar PrP accumulations
	VV1	dementia at onset, later ataxia and extrapyramidal disorders	spongiosis, gliosis and loss of innervation of the cortical structures while omitting the brainstem and the cerebellum
vCJD	MM2b	psychiatric, dysaesthesia, ataxia, later dementia	spongiosis, gliosis and loss of innervation as well as 'florid' plaques