Skip to main content

Table 5 Severity score index for patients with onset between 9 and 12 months

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

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
  1. Displays clinical data for the 12 patients with disease onset between 9 and 12 months of life. A 1 indicates that the patient presented with the clinical marker at their first evaluation and a 0 indicates that the patient did not present with the clinical marker at their first evaluation. Values in the total column represent the patient’s severity score. Patients scoring a 6 or above are expected to progress more rapidly than patients with a score of 0–5