| Positive features |
| Definite ALS |
| LMN and UMN signs in three to four regions |
| Evidence of progression |
| Probable ALS |
| LMN and UMN signs in at least tworegions with UMN above LMN signs and evidence of progression |
| Possible ALS |
| LMN and UMN in one region |
| UMN in two regions |
| LMN above UMN signs |
| LMN and UMN signs but no evidence of Progression |
| Suspected ALS |
| LMN signs in two to three regions |
| Negative features |
| Findings inconsistent with diagnosis of ALS |
| Neuroimaging, EMG, clinical or other evidence of an alternative disease explaining signs or symptoms |
| Lack of progression to other body regions |
| Cognitive decline |
| Sphincter abnormalities |
| Sensory dysfunction |
| Visual decline |