Table 2.

Anatomical distribution and severity of FUS-immunoreactive pathology in aFTLD-U cases

	Case 1	Case 2	Case 3	Case 4	Case 5	Case 6	Case 7	Case 8	Case 9	Case 10	Case 11	Case 12	Case 13	Case 14	Case 15
Frontotemporal neocortex	++	++	++	++	++	+++	+	+	++	+	+++	+	+	++	+
Hippocampus	+++	NA	++	++	+++	+++	++	+	++	++	+++	++	+++	++	+++
Striatum	++	++	+	++	++	++	+	+	++	+	++	NA	+	NA	++
Lower motor neurons	+	++	++	NA	++	+	+	NA	NA	+	+	NA	++	+	NA

Grading: −= absent; += mild; ++ = moderate; +++ = severe; NA = not available.

Score is aggregate of all FUS-positive pathology, including neuronal cytoplasmic inclusions, neuronal intranuclear inclusions, glial inclusions and dystrophic neurites.