Mutations in SQSTM1 were found in 0.9 to 3% of cases of ALS and FTD(familial and sporadic), and in less than 5% of the familial MSP.89,93,95 The onset ofsymptoms in FTD ranged from between 48 and 73 years.89,93SQSTM1 mutations have been identified in rare patients diagnosedwith nfvPPA, svPPA, PSP and CBS, but the pathogenicity of these mutations has yet tobe confirmed.96 Here, CBS is linked to frontotemporal dementia.