Review of studies investigating cognitive changes in presymptomatic C9+ vs C9− individuals
| Study | N | Mean age (SD) | Mean estimated time to disease onset (SD) | Mean age at family onset (SD) | Discriminant tests | Non-discriminant tests |
| Panman et al 2019 | 12 | 49.7 (12.4) | – | – | – |
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| Bertrand et al 2018 | 41 | 39.8 (11.1) | 19.3 (11.2) | – |
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| Popuri et al 2018 | 15 | 42.6 (11.3) | 13.7 (11.2) | – | – |
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| Papma et al 2017 | 18 | 45.8 (13.8) | – | 53.0 (5.3) |
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| Lee et al 2016 | 15 | 43.7 (10.2) | 8.2 (11.0) | 51.8 (5.3) |
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| Walhout et al 2015 | 16 | 45.5 (14.7) | – | – | – |
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ALS, amyotrophic lateral sclerosis; BDI, Beck Depression Inventory; BNT, Boston Naming Test; CATS, Comprehensive Affective Testing System; CBI-R, Cambridge Behavioural Inventory–Revised; CVLT, California Verbal Learning Test; FAB, Frontal Assessment Battery; FBI, Frontal Behavior Inventory; GDS, Geriatric Depression Scale; IRI, Interpersonal Reactivity Index; LDST, Letter Digit Substitution Test; MDRS, Mattis Dementia Rating Scale; Mini-SEA, Mini Social Cognition and Emotional Assessment; MMSE, Mini-Mental State Examination; NPI, Neuropsychiatric Inventory; RAVLT, Rey Auditory Verbal Learning Test; VOSP, Visual Object and Space Perception Battery.