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had a profile of amnesic mild cognitive impairment and 5 (1 VUS) had cognitively-normal profile. At follow up, three ALSbi patients (one with VUS) developed frank frontotemporal dementia (FTD) and one ALSbi developed cognitive impairment (ALScbi). The most common behavioural changes were apathy, mental rigidity and irritability. ConclusionsOur findings demonstrate that SOD1 patients have early behavioural impairment more commonly than previously reported, which might increase the risk to develop FTD.