Table 1 Clinical, molecular and pathological features of the iatrogenic CJD patients of this study and sporadic CJDMM1

1 (index case)

2

3

4

Ataxia, weight loss and insomnia.

Ataxia and slurred speech.

Left leg numbness with broad based gait.

Visual changes and ataxic gait.

Rapidly progressive cognitive decline; occasionally cortical visual disturbances, ataxia, and myoclonus.

50/male

54/male

40/female

39/female

65.5^b (42–91)^c

14

2

2

4

3.9^b (1–18)^c

38^d

41.5^d

26.3^d

6^e

1

1

1

1

1

Met/Met

Met/Met

Met/Met

Met/Met

Met/Met

+++

-

-

-

-

+

-

-

-

-

+++

-

-

-

-

++/−

−/−

−/−

−/−

−/−

++/+++

−/−

−/−

−/−

−/−

Severe in subcortical regions; preferentially in layers IV-VI of cerebral cortex (cc); cerebellar molecular layer, focal.

Less severe in occipital than frontal cc; entorhinal cc and basal ganglia spared; occasionally most severe in deep layers of the cc; cerebellar molecular layer, focal.

More severe in occipital than frontal cc; entorhinal cc and basal ganglia affected; occasionally most severe in deep layers of cc; cerebellar molecular layer, ubiquitous.

All layers of cc affected; more severe in occipital than frontal cc; entorhinal cc and basal ganglia affected; cerebellar molecular layer, focal.

All layers of cc affected; more severe in the occipital than frontal cc; entorhinal cc and basal ganglia affected; cerebellar molecular layer, focal.

PrP “stellate”, kuru plaques, plaque-like; occasionally “brush stroke-like”.

“brush stroke-like”

“brush stroke-like”

“brush stroke-like”

“brush stroke-like”