Table 2 Amyotrophy in Creutzfeldt-Jakob disease – reported patients

Allen et al., 1971 [9]

M

56

13

◦ Asymmetrical weakness and wasting of the upper limbs

At onset

First sign at clinical presentation

◦ Frontal cortex neurodegeneration, marked gliosis

◦ Generalized fasciculations

◦ Spongiform change of temporal lobes

◦ Memory loss

◦ Loss of anterior horn cells with accompanied gliosis

◦ Confabulation and constructional ataxia

Traub et al., 1977 [10]

F

35

12

◦ Memory loss

ND

10 months after clinical presentation

◦ Severe spongiform change, astrocytosis in the putamen, caudate nucleus and amygdala

◦ Diplopia

◦ Degeneration of lateral corticospinal tracts

◦ Neuronal loss of anterior horn cells

Traub et al., 1977 [10]

M

61

9

◦ Memory loss

ND

6 months after clinical presentation

◦ Neurodegeneration of cerebral cortex

◦ Confusion

◦ Neuronal loss and astrocytosis of anterior horn cells

Nowacki et al., 2000 [8]

M

82

2

◦ Memory loss

3 weeks

4 weeks after symptomatic onset

◦ Extensive spongiform change and neuronal loss

◦ Confusion

◦ Loss of anterior horn cells

Esteban et al., 2001 [4]

M

62

18

◦ Memory loss

4 months

10 months after symptomatic onset

◦ Neocortical and hipppocampal spongiosis

◦ Severe neuronal loss with gliosis in the amygdala

◦ Loss of anterior horn cells

Kovacs et al., 2002 [7]

M

62

8

◦ Numbness in left foot

5 months

7 months after symptomatic onset

◦ Extensive spongiform change, neuronal loss and astrocytosis

◦ Reduced neuronal density in ventral horn

Niewiadomska et al., 2002 [5]

M

67

10.5

ND

ND

ND

ND

Niewiadomska et al., 2002 [5]

M

65

24

ND

ND

ND

ND

Niewiadomska et al., 2002 [5]

M

60

9

ND

ND

ND

ND

Panegyres et al., 2013

M

70

1.2

◦ Limb weakness and falls

At onset

First sign at clinical presentation

◦ Extensive spongiform change, neuronal loss and astrocytosis in the neocortex, with 12F10 synaptic immunopositivity in cerebral cortex, thalamus, cerebellar cortex and brainstem motor neurons.