Transmissible Spongiform Encephalopathies Affecting Humans

Table 1

Pathological features in CJSs [35].

Characteristic	Typical sporadic CJD	Variant CJD
Median age at death	68 years	28 years
Median duration of illness	4-5 months	13-14 months
Clinical signs and symptoms	Dementia: early neurologic signs	Prominent psychiatric/behavioral symptoms: painful dysesthesias: delayed neurologic signs
Periodic sharp waves on electroencephalogram	Often present	Often absent
Signal hyperintensity in the caudate nucleus and putamen on diffusion-weighted and FLAIR MRI	Often present	Often absent
Pulvinar signon MRI	Not reported	Present in >75% of cases
Immunohistochemical analysis of brain tissue	Variable accumulation	Marked accumulation of protease-resistant prion protein
Presence of agent in lymphoid tissue	Not readily detected	Readily detected
Increased glycoform ratio on immunoblot analysis of protease-resistant prion protein	Not reported	Marked accumulation of protease-resistant prion protein
Presence of amyloid plaques in brain tissue	May be present	May be present