Table II.
Phenotype classification based on clinically imputed anatomy of neuropathology.
| CNS Anatomical Region Involved* |
Innervated Peripheral Body Region Involved* |
|||||
|---|---|---|---|---|---|---|
| UMN | LMN | Fronto-temporal regions |
Bulbar muscles |
Limb muscles |
Higher cortical function & behavior |
|
| Based on Level of involvement | ||||||
| ALS | ++ | ++ | +/ − | ++ | ++ | +/− |
| PLS | ++++ | +/ − | +/ − | ++ | ++ | +/− |
| PMA | +/− | ++++ | +/− | +/ − | ++++ | +/− |
| Based on Body Region of involvement | ||||||
| Bulbar ALS | +/− | ++++ | +/− | ++++ | +/− | +/− |
| Pseudobulbar ALS | ++++ | +/ − | +/ − | ++++ | +/− | +/− |
| Limb-onset ALS | ++ | ++ | +/ − | +/ − | ++++ | +/− |
| Limb-variants | +/− | ++++ | +/− | +/ − | ++++ | +/− |
| Mill’s variant | ++++ | +/ − | +/− | +/ − | ++++ | +/− |
| Associated cognitive changes | ||||||
| FTD or behavioral/cognitive impairment | +/− | +/ − | ++++ | +/ − | +/− | ++++ |
*
+/− possible but not typical; ++ typical and to variable degree; ++++ primary feature.