Table 2 Amyotrophy in Creutzfeldt-Jakob disease – reported patients
From: A patient with Creutzfeldt-Jakob disease presenting with amyotrophy: a case report
| Reference | Gender | Age at onset | Disease duration (months) | Initial symptoms | Duration of symptoms before presentation | Presence of amyotrophy | Neuropathology |
|---|---|---|---|---|---|---|---|
| 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. |