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Table 2 Signs and symptoms of diseases in respect to age of onset

From: A prospective natural history study of Krabbe disease in a patient cohort with onset between 6 months and 3 years of life

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)
  1. Displays common clinical indicators of disease progression observed by the physician or reported by parents at evaluation. Children are separated into age groups based on age at the onset of first symptoms