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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.
  1. ND not described.
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