Table 1 Symptom incidence in each age group and cohort as a whole
From: Early progression of Krabbe disease in patients with symptom onset between 0 and 5 months
0-3 months (n = 16) | 4-6 months (n = 28) | 7-9 months (n = 36) | 10-12 months (n = 21) | 13-17 months (n = 19) | 18-23 months (n = 11) | >24 months (n = 16) | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Symptom | N | Mean | N | Mean | N | Mean | N | Mean | N | Mean | N | Mean | N | Mean |
Reflux | 12 | 17% | 26 | 62% | 25 | 84% | 18 | 61% | 8 | 50% | 5 | 40% | 11 | 45% |
Feeding Difficulties or swallowing difficulties | 14 | 7% | 28 | 79% | 33 | 97% | 21 | 90% | 16 | 88% | 8 | 100% | 12 | 100% |
Required Suctioning | 12 | 0% | 19 | 11% | 28 | 50% | 18 | 50% | 16 | 81% | 11 | 91% | 15 | 87% |
Diarrhea | 10 | 0% | 20 | 25% | 20 | 10% | 16 | 6% | 12 | 0% | 6 | 0% | 12 | 17% |
Constipation | 10 | 10% | 21 | 38% | 29 | 59% | 18 | 56% | 16 | 63% | 10 | 70% | 16 | 75% |
Visual Tracking Difficulty | 14 | 7% | 28 | 57% | 33 | 58% | 19 | 68% | 19 | 89% | 11 | 64% | 16 | 88% |
Jerky eye movements/eye fluttering | 13 | 0% | 17 | 12% | 26 | 27% | 19 | 32% | 14 | 29% | 9 | 78% | 13 | 54% |
Nystagmus | 12 | 0% | 17 | 12% | 24 | 0% | 18 | 17% | 18 | 22% | 10 | 20% | 14 | 21% |
Hip asymmetry | 12 | 0% | 15 | 7% | 25 | 12% | 21 | 24% | 19 | 47% | 11 | 55% | 15 | 87% |
Scoliosis | 10 | 0% | 16 | 13% | 28 | 14% | 20 | 40% | 17 | 65% | 10 | 70% | 16 | 94% |
Axial Hypotonia | 12 | 17% | 23 | 65% | 30 | 83% | 21 | 100% | 19 | 100% | 11 | 100% | 15 | 100% |
Abnormal DTRs | 12 | 17% | 26 | 88% | 31 | 94% | 21 | 95% | 19 | 100% | 11 | 100% | 15 | 100% |
Clasped Thumb/Hand Fisting | 12 | 33% | 26 | 92% | 32 | 94% | 20 | 85% | 18 | 78% | 10 | 60% | 15 | 40% |
Appendicular Spasticity | 13 | 8% | 24 | 100% | 34 | 97% | 20 | 90% | 19 | 74% | 11 | 64% | 16 | 75% |
Clinical Seizures | 10 | 0% | 18 | 6% | 25 | 8% | 20 | 15% | 16 | 19% | 9 | 33% | 15 | 33% |
Staring Episodes | 10 | 0% | 17 | 41% | 27 | 48% | 17 | 41% | 14 | 64% | 9 | 89% | 12 | 92% |
Shallow Breathing or Cheyne-Strokes | 12 | 0% | 24 | 17% | 30 | 37% | 21 | 48% | 19 | 79% | 11 | 82% | 16 | 88% |
Apneic Episodes | 12 | 0% | 17 | 18% | 26 | 23% | 19 | 32% | 17 | 59% | 10 | 60% | 16 | 63% |
Poor Peripheral Perfusion | 11 | 0% | 18 | 0% | 24 | 4% | 18 | 17% | 18 | 44% | 10 | 60% | 14 | 71% |
Temperature Instability | 14 | 0% | 28 | 0% | 34 | 6% | 21 | 14% | 19 | 32% | 11 | 55% | 16 | 69% |
Abnormal Pupillary Response | 11 | 0% | 23 | 22% | 30 | 43% | 19 | 58% | 19 | 89% | 11 | 100% | 14 | 100% |