Table 5 Severity score index for patients with onset between 9 and 12 months
Age of Onset | Age of Initial Evaluation | Rate of Disease Progression | Transplanted? | Reflux | Constipation | Feeding Difficulties | Tracking Difficulties | Abnormal Pupillary Response | Staring Episodes | Seizures | Clonus | Abnormal DTR |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
12 | 12 | Slower | Yes | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
12 | 23 | Slower | Yes | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
10 | 17 | Slower | Yes | 0 | 1 | 0 | 0 | 0 | 0 | 0 | 0 | 1 |
12 | 21 | Slower | Yes | 1 | 0 | 1 | 0 | 1 | 0 | 0 | 0 | 0 |
9 | 14 | Slower | No | 1 | 1 | 1 | 0 | 0 | 0 | 0 | 0 | 1 |
10 | 32 | Rapid | No | 0 | 0 | 1 | 0 | 1 | 1 | 0 | 1 | 1 |
10 | 13 | Rapid | No | 1 | 1 | 1 | 1 | 0 | 0 | 0 | 1 | 1 |
12 | 14 | Rapid | No | 1 | 1 | 1 | 0 | 1 | 0 | 1 | 1 | 1 |
12 | 38 | Rapid | No | 1 | 1 | 1 | 1 | 0 | 1 | 1 | 1 | 1 |
12 | 17 | Rapid | No | 0 | 1 | 1 | 1 | 1 | 1 | 1 | 1 | 1 |
11 | 35 | Rapid | No | 1 | 1 | 1 | 1 | 1 | 1 | 1 | 1 | 1 |
9 | 14 | Rapid | No | 1 | 1 | 1 | 1 | 1 | 1 | 1 | 1 | 1 |