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[Preprint]. 2023 Aug 10:rs.3.rs-3183113. [Version 1] doi: 10.21203/rs.3.rs-3183113/v1

Figure 3. Comparison of clinical, genetic and pathological characteristics across subtypes.

Figure 3

(a) number of clinical phenotypes, cases carrying genetic pathogenic variants, symptom onset sites and TDP-43 types assigned to each subtype. Comparison of (b) SuStaIn stage, (c) disease duration, (d) diagnostic delay, (e-l) cognitive scores across subtypes in all individuals, and (m) PUMNS, (n) ALSFRS-R, (o) progression index, (p) King’s stage across subtypes in individuals with ALS/ALS-FTD. *p value < 0.05, **p value < 0.01, ***p value < 0.001, ****p value < 0.0001.

S0 Normal-appearing group, S1 Prefrontal/Somatomotor-predominant subtype, S2 Limbic-predominant subtype, MMSE Mini-Mental Status Examination, ECAS Edinburgh Cognitive Assessment Scale, PBAC Philadelphia Brief Assessment of Cognition, BNT Boston naming test, PUMNS Penn Upper Motor Neuron Score, ALSFRS-R Revised ALS Functional Rating Scale, LMN lower motor neuron, UMN upper motor neuron.