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Table 1 Neurocognitive tests employed in patients with neuronopathic MPS according to age

From: Neurobehavioral phenotypes of neuronopathic mucopolysaccharidoses

 

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)

[60,61,62]

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, 60,61,62]

 Treatment outcome

MPS I (HSCT)

MPS I (HSCT)

MPS I (HSCT)

[23, 25, 60,61,62]

  1. 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
  2. a Range of age referred according to manual