Table 5

Developmental assessment in 10 patients older than 2.5 years of age

Median (range) at M0Median (range) at M12Median (range) change M0 vs. M12N of improved ptsP***N of affected at M0/M12
Total IQ (n = 10)60 (40–92)61.5 (40–94)0.5 (−13 to 6)00.956/6
Verbal IQ (n = 6)80 (45–106)83 (47–85)*−1 (−21 to 4)*012/2
Performance IQ (n = 6)66 (47–85)79 (45–100)*1 (−7 to 15)*00.633/2
Verbal Comprehension Index (n = 8)61 (45–96)*75.5 (45–99)0 (−7 to 15)*10.944/3
Perceptual Reasoning Index (n = 8)60 (45–90)*57.5 (47–99)1 (0 to 21)*00.134/3
Working Memory Index (n = 8)71 (50–98)*83.5 (50–100)2 (0 to 18)*10.133/3
Processing Speed Index (n = 9)75 (50–114)**71 (50–108)0 (−6 to 11)**10.883/4
  • Tests used: Wechsler Preschool and Primary Scale of Intelligence–Revised for children and Wechsler Intelligence Scale for Children–Fourth Edition for patients younger than 16 years and 11 months old at inclusion (patient nos. 10, 11, 12, 13, 15, 16, 17, and 18) and Wechsler Adult Intelligence Scale–Third Edition for children older than 17 years old (patient nos. 9 and 14). M0, baseline (just before starting SU therapy); M12, 12 months after starting SU therapy; pts, patients.

  • *One patient was not evaluated.

  • **Two patients were not evaluated.

  • ***Paired Wilcoxon test on continuous scores.