Table 2 Signs and symptoms of diseases in respect to age of onset
Clinical Indicators of Disease Progression | All Ages n/N (%) | Age at Onset | |
|---|---|---|---|
≤12 Months n/N (%) | > 12 Months n/N (%) | ||
Abnormal protective reflexes | 29/32 (91) | 23/23 (100) | 6/9 (67) |
Axial hypotonia | 29/32 (91) | 23/23(100) | 6/9 (67) |
Appendicular hypertonia | 28/32 (88) | 20/23 (87) | 8/9 (89) |
Abnormal deep tendon reflexes | 22/29 (76) | 17/22 (77) | 5/7 (71) |
Constipation | 22/32 (69) | 16/23 (70) | 6/9 (77) |
Feeding difficulty | 23/32 (72) | 20/23 (87) | 3/9 (33) |
Thumb clasping or hand fisting | 19/32 (59) | 18/23 (78) | 1/9 (11) |
Asymmetric tonic neck reflex | 18/32 (56) | 17/23 (74) | 1/9 (11) |
Babinski reflex | 18/32 (56) | 15/23 (65) | 3/9 (33) |
Abnormal pupillary response | 18/32 (56) | 13/23 (56) | 5/9 (56) |
Gastroesophageal reflux | 17/32 (53) | 15/23 (65) | 2/9 (22) |
Open mouth posture | 15/32 (47) | 13/23 (56) | 2/9 (22) |
Slow weight gain | 13/32 (41) | 11/23 (48) | 2/9 (22) |
Failure to thrive | 9/32 (28) | 9/23 (39) | 0/9 (0) |
Appendicular hypotonia | 2/32 (6) | 1/23 (4) | 1/9 (11) |