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Table 2 Neurocognitive test results in MPS III patients with a mild- or non-neuropathic phenotype

From: The attenuated end of the phenotypic spectrum in MPS III: from late-onset stable cognitive impairment to a non-neuronopathic phenotype

PatientAge at Testing (years)TestDomainIndex scores95% CI
1.1 a64WAIS-IV short formVCI111105–116
  PRI8575–94
2.1 a55WAIS-IV short formVCI9388–99
  PRI125116–131
5.1 a54WAIS-IV short formVCI10094–106
  PRI116107–122
6.2 c20WAIS-IVFSIQ b7975–85
  VCI109103–114
  WMI8074–89
  PRI7266–81
  PSI5954–73
7.218WAIS-IVFSIQ b9691–101
  VCI113107–118
  WMI9285–100
  PRI10092–108
  PSI7669–88
8.350WAIS-IV short formVCI9388–99
10.4 c26WAIS-IIIFSIQ b6865–73
  VCI7974–85
  WMI6863–77
  PRI6156–72
  PSI5551–69
11.5 c42WAIS-IVFSIQ5047–55
  VCI6662–73
  WMI5551–64
  PRI5248–61
  PSI5047–63
Cases from literature
 1 [17]31WAISFSIQ88NK
 2 [17]36WAISFSIQ75NK
  1. Neurocognitive test scores are based on a mean of 100 with a SD of 15.
  2. Abbreviations: FSIQ full scale IQ; NK not known; PRI perceptual reasoning index; PSI processing speed index; VCI verbal comprehension index; WAIS Wechsler Adult Intelligence Scale; WMI working memory index.
  3. a VCI and PRI estimated using the proration method [25], extrapolating the scores on 2 subtest within each index.
  4. b FSIQ should be interpreted with caution given significant discrepancies between the index scores for the different components.
  5. c Functions with a mild neurocognitive impairment.