Table 3.
Cognitive and behavioral features of C9Pos and C9Neg patients
| C9Pos | C9Neg | P valuea | |||||||
|---|---|---|---|---|---|---|---|---|---|
| n | % | Mean | Median | n | % | Mean | Median | ||
| executive | 34.0 | 35.5 | 33.9 | 36.0 | ns | ||||
| fluency | 17.1 | 20.0 | 16.1 | 18.0 | ns | ||||
| language | 23.5 | 24.4 | 23.3 | 24.0 | ns | ||||
| ALS-specific | 74.6 | 79.0 | 73.4 | 78.0 | ns | ||||
| memory | 16.5 | 17.5 | 15.0 | 16.0 | ns | ||||
| visuospatial | 11.1 | 11.0 | 11.4 | 12.0 | ns | ||||
| ALS-nonspecific | 27.6 | 29.0 | 26.4 | 27.0 | ns | ||||
| ECAS total score | 102.1 | 107.0 | 100.1 | 104.0 | ns | ||||
| dishinibition | 5 | 29.4 | 8 | 4.7 | 1.6 × 10–4 | ||||
| apathy/inertia | 5 | 29.4 | 167 | 37.7 | ns | ||||
| loss of sympathy/empathy | 4 | 23.5 | 23 | 13.8 | ns | ||||
| perseverative/stereotyped/compulsive/ritualistic behavior | 0 | 0.0 | 3 | 1.2 | ns | ||||
| hyperorality/altered food preferences | 0 | 0.0 | 4 | 2.4 | ns | ||||
| ECAS behavioral symptoms | 0.8 | 1.0 | 0.6 | 0.0 | ns | ||||
| FBI-A | 2.5 | 2.0 | 2.3 | 1.0 | ns | ||||
| FBI-B | 2.2 | 2.0 | 1.0 | 0.0 | 0.009 | ||||
| FBI | 4.7 | 5.0 | 3.4 | 2.0 | ns | ||||
| BDI-II cognitive-affective | 3.7 | 4.0 | 6.6 | 5.0 | 0.018 | ||||
| BDI-II somatic | 5.5 | 5.0 | 7.6 | 7.0 | 0.042 | ||||
| BDI-II | 9.2 | 7.0 | 14.2 | 13.0 | 0.015 | ||||
| STAI 1 | 45.2 | 42.0 | 50.8 | 49.0 | 0.008 | ||||
| STAI 2 | 45.1 | 43.0 | 50.2 | 49.0 | 0,033 | ||||
ns, not significant; C9Pos, patients carrying C9orf72 repeat expansion; C9Neg, patients without C9orf72 repeat expansion; ECAS, Edinburgh Cognitive and Behavioural ALS Screen; FBI, Frontal Behavioural Inventory; BDI-II, Beck Depression Inventory-II; STAI, State-Trait Anxiety Inventory
aComparison between C9Pos and C9Neg patients