Table 2.
Examination and investigation findings
| Case | ALS onset | Amyotrophy | UMN signs | Limb weakness | Bulbar weakness | Imaging | EMG/NCS |
|---|---|---|---|---|---|---|---|
| 1 | Unknown | – | – | ++ | + | – | – |
| 2 | Unknown | – | + | ++ | + | – | – |
| 3 | Bulbar | + | + | † † | + | – | – |
| 4 | Lower limb | + | + | ++ | – | Generalised atrophy (CT) | Chronic partial denervation |
| 5 | Bulbar | – | + | † † | † † | Normal (CT) | – |
| 6 | Upper limb | + | + | ++ | + | Parietal and occipital atrophy (MRI) | Reduced CMAPs fibrillation potentials |
| 7 | Limb | – | + | † | – | Generalised atrophy (CT) | – |
| 8 | Limb | + | + | † † | † | Bitemporal atrophy (MRI) | Normal early in disease course |
| 9 | Bulbar | – | + | + | ++ | Normal (MRI) | Denervation |
UMN Upper motor neuron, EMG/NCS electromyography and nerve conduction studies, CMAPs compound muscle action potentials, ++: main presenting feature,+: minor presenting feature, ††: late presenting features