Table 1.
Regions of the brain involved in pTDP-43 staging system in ALS and bvFTD
| Regions of interest | ALS | bvFTD |
|---|---|---|
| Grey matter | ||
| Precentral gyrus (motor cortex) | Stage 1 | Pattern III |
| Brainstem | ||
| Hypoglossal nucleus | Stage 1 | Pattern III |
| Precerebellar nuclei/red nucleus | Stage 2 | Pattern II |
| Middle frontal gyrus | Stage 2 | Pattern II |
| Cingulate—anterior | Stage 2/3 | Pattern II/III |
| Postcentral gyrus (somatosensory cortex) | Stage 3 | Pattern III |
| Orbitofrontal cortex | ||
| Orbital gyrus | Stage 3 | Pattern I |
| Gyrus rectus | Stage 3 | Pattern I |
| Striatum | Stage 3 | Pattern II |
| Hippocampal formation | Stage 4 | Pattern II |
| Occipital pole | Not affected | Pattern IV |
| White matter tracts | ||
| Corticospinal tract | Stage 1* | Pattern III* |
| Cingulum | Not affected [27] | Affected* |
| Fornix | Not assessed | Affected* |
| Corpus callosum | Affected* | Affected* |
The table highlights brain regions that have been described in the post mortem four stage hypothesis of pTDP-43 [12, 25], or demonstrated to be involved in the disease process in vivo
* White matter tract changes demonstrated with in vivo MR imaging, however, independent of pTDP-43 validation or in the absence of pathology [27, 39]