Answers

1. Neuroimages of the brain showed asymmetric parietal and perisylvian atrophy. Axial T1-weighted (a), axial fluid-attenuated inversion recovery (b), axial T2-weighted gradient-echo (c), coronal T2-weighted (d), and sagittal fluid-attenuated inversion recovery (d and e) images of the brain MRI [Figure 2].
2. Probable nonfluent/agrammatic variant of primary progressive aphasia.

Corticobasal degeneration (CBD) is a rare and progressive neurodegenerative disease, which is probably related to pathological aggregation of tau protein in neurofibrillary or gliofibrillary tangles in the human brain. It was first described in 1967 and later recognized as a polymorphous disease due to its complexity. In this context, the classical symptoms, asymmetric motor features associated with higher cortical function, were associated with multiple histological presentations. A number of clinical criteria for CBD have already been proposed, but none was validated. In 2013, a review of pathologically confirmed cases of CBD, different phenotypes associated with CBD pathology, and these phenotypes were used to create two sets of consensus criteria the probable and the possible [Table 1].^[1]

Figure 2: Neuroimages of the brain showed asymmetric parietal and perisylvian atrophy (arrows). Axial T1-weighted (a), axial fluid-attenuated inversion recovery (b), axial T2-weighted gradient-echo (c), coronal T2-weighted (d), and sagittal fluid-attenuated inversion recovery (e and f) images of the brain MRI Click here to view

Table 1: Corticobasal degeneration clinical criteria - systematic review (2013) Click here to view

Another interesting fact is that in the early stages, brain imaging may be normal. As disease progresses, abnormalities such as asymmetric cortical atrophy are observed in almost 50% of the subjects. In this way, focal atrophy predominantly involves the posterior frontal and parietal regions, and atrophy of the corpus callosum is also commonly seen.^[2]