Frequency of cognitive impairment classified according to the consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in ALS9
| ATXN2+ (n=25) | ATXN2- (n=903) | |
| Cognitively normal ALS | 10 (40%) | 483 (53.5%) |
| ALSbi | – | 72 (8%) |
| ALSci | 7 (28%) | 164 (18.2%) |
| ALScbi | 1 (4%) | 63 (7%) |
| ALS-FTD | 7 (28%) | 121 (13.4%) |
In patients with ALS with ATXN2 PolyQ intermediate number of repeats ≥31 (ATXN2+) compared with patients with PolyQ ≤30 (ATXN2-). ALS-FTD was significantly more frequent in ATXN2+ patients (p=0.037).
ALS, amyotrophic lateral sclerosis; ALSbi, ALS with behavioural impairment; ALScbi, ALS with cognitive and behavioural impairment; ALSci, ALS with cognitive impairment; ALS-FTD, ALS with comorbid FTD.