Table 2.
Relative brain distribution of poly‐GA immunopositive neuronal cytoplasmic inclusions (dipeptide repeat proteins, DPR), and TDP‐43 immunopositive neuronal cytoplasmic inclusions and/or neurites in the three Newcastle cases of frontotemporal lobar degeneration (FTLD) bearing hexanucleotide expansions in C9ORF72 gene, and range of scores for DPR and TDP‐43 pathology in the 13 Manchester cases with which these are compared
| Brain region | Newcastle case 1 | Newcastle case 2 | Newcastle case 3 | Manchester cases | ||||
|---|---|---|---|---|---|---|---|---|
| DPR | TDP | DPR | TDP | DPR | TDP | DPR | TDP | |
| Frontal cortex | 3 | 0.5 | 1 | 0.5 | 2 | 0.5 | 2–4 | 1–3 |
| Temporal cortex | 3 | 0.5 | 2 | 0.5 | 2 | 1 | 2–4 | 1–3 |
| Cingulate cortex | 2 | 0.5 | 1 | 0.5 | 2 | 0.5 | 2–4 | 1–3 |
| Insular cortex | 3 | 0 | 1 | 0 | 2 | 0 | 2–4 | 0 |
| Entorhinal cortex | 2 | 0 | 2 | 0 | 0 | 0 | 2–4 | 0 |
| Fusiform gyrus | 2 | 0 | 2 | 0 | 1 | 0 | 2–4 | 0 |
| Parietal cortex | 2 | 0 | 1 | 0 | 2 | 0 | 2–4 | 0 |
| Occipital cortex | 1 | 0 | 2 | 0 | 1 | 0 | 2–4 | 0 |
| Hippocampus dentate gyrus | 2 | 0.5 | 2 | 0.5 | 2 | 0.5 | 2–4 | 1–3 |
| Hippocampus CA4 region | 2 | 0 | 3 | 0 | 2 | 0 | 2–4 | 0 |
| Hippocampus CA3 region | 2 | 0 | 2 | 0 | 1 | 0 | 2–3 | 0 |
| Hippocampus CA2 region | 0.5 | 0 | 1 | 0 | 1 | 0 | 1–2 | 0 |
| Putamen | 0.5 | 0 | 0 | 0 | na | na | 0–1 | 0 |
| Thalamus | 1 | 0 | na | na | na | na | 1–3 | 0 |
| Cerebellar granule cells | 4 | 0 | 3 | 0 | 4 | 0 | 3–4 | 0 |
| Purkinje cells | 0 | 0 | 0 | 0 | 0.5 | 0 | 0–1 | 0 |
| Dentate nucleus | 0.5 | 0 | 0 | 0 | 0 | 0 | 0–1 | 0 |
na, tissue not available.