Table 1.
Neurocognitive tests employed in patients with neuronopathic MPS according to age
| Tests employed | References | |||
|---|---|---|---|---|
| Age ≤ 36 months | BSID-III | MSEL | GMDS-III | |
| Rangea | 0–42 months | 0–68 months | 0–72 months | |
| Natural history, MPS characteristic studies | MPS I, II, III | MPS I, II, III | MPS III | [6, 19, 37, 38, 60] |
| Treatment outcome | MPS I (HSCT), MPS II (ERT); MPS IIIA (IT-ERT) | MPS I (HSCT) | MPS I (HSCT, ERT), MPS II (HSCT, ERT), MPS III (HSCT) | [18, 21, 29, 60] |
| Age > 36 months, ≤ 6 years | KABC-II | Leiter-III | WPPSI-IV | |
| Rangea | 3–18 years | 3–75 years | 2.6–7.7 years | |
| Natural history, MPS characteristic studies | MPS III | – | MPS I, II | [29, 33, 48, 60, 61] |
| Treatment outcome | – | MPS I ((HSCT), MPS III (HSCT) | MPS I (HSCT) | [60–62] |
| Age > 6 years and adulthood | WISC-IV | WAIS-IV | WASI | |
| Rangea | 6.0–16.11 years | 16–90 years | 6–89 years | |
| Natural history, MPS characteristic studies | MPS II, III | MPS I, II, III | MPS I, II | [18, 31, 40, 60–62] |
| Treatment outcome | MPS I (HSCT) | MPS I (HSCT) | MPS I (HSCT) | [23, 25, 60–62] |
BSID Bayley Scales of Infant Development, ERT enzyme replacement therapy, GMDS Griffiths Mental Development Scales, HSCT hematopoietic stem cell transplantation, IT intrathecal, KABC Kaufman Assessment Battery for Children, MPS mucopolysaccharidosis, MSEL Mullen Scales of Early Learning, WAIS Wechsler Adult Intelligence Scale, WASI Wechsler Abbreviated Scale of Intelligence, WISC Wechsler Intelligence Scale for Children, WPPSI Wechsler Preschool and Primary Scale of Intelligence
a Range of age referred according to manual