It was recently reported by analyzing an ALS registry that ATXN2 intermediate repeat expansions are not only associated with shorter survival and spinal onset but also with a faster time to diagnosis from onset of symptoms, faster decline of ALSFRS-R (Revised Amyotrophic Lateral Sclerosis Functional Rating Scale) score and with the presence of comorbid frontotemporal dementia [54]. Here, ATXN2 is linked to frontotemporal dementia.