Clinical characteristics
| Sex (M:F) | 14:6 | |
|---|---|---|
| Mean age at onset (range)(y) | 62 | (44–73) |
| Duration of symptoms (mean (range)y) | 6 | (1–13) |
| Patients given diagnosis of ALS | 7 | |
| Cramps | 0 of 10 | |
| Symptoms at onset (n (%)): | ||
| Legs | 13 of 20 | 65 |
| Arms | 6 of 20 | 30 |
| Dysphagia | 2 of 20 | 10 |
| Asymmetry at onset (n (%)) | 5 of 20 | 25 |
| Finger flexor weakness or atrophy (n (%)) | 13 of 20 | 65 |
| Quadriceps weakness or atrophy (n (%)) | 17 of 20 | 85 |
| Neck weakness (n (%)) | 3 of 11 | 27 |
| Bulbar symptoms (n (%)) | 6 of 19 | 32 |
| Facial weakness (n (%)) | 7 of 18 | 39 |
| Mean serum CK (X upper limit of normal) | 4.1 | 0.7–12.9 |
| Response to IVIG (minimal) | 4 of 7 | |
| Response to prednisone | 2 of 9 | |
| Response to azathioprine (imuran) | 0 of 3 |