“…These include sporadic and familial cases with both juvenile and adult-onset [1, 9, 12, 16
17, 33, 36]. Following the report that the BIBD subtype of FTLD was characterized by FUS-ir pathology [25], several cases of ALS with BI were re-evaluated [3, 6, 11, 15, 23, 34, 35, 39]. These studies demonstrated that the BI in cases of ALS are also consistently FUS-ir and that most [3, 11, 15, 34, 35, 39], but not all cases [11, 6, 23], are associated with a FUS mutation.…”