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

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

Patient	Age at Testing (years)	Test	Domain	Index scores	95% CI
1.1 ^a	64	WAIS-IV short form	VCI	111	105–116
1.1 ^a			PRI	85	75–94
2.1 ^a	55	WAIS-IV short form	VCI	93	88–99
2.1 ^a			PRI	125	116–131
5.1 ^a	54	WAIS-IV short form	VCI	100	94–106
5.1 ^a			PRI	116	107–122
6.2 ^c	20	WAIS-IV	FSIQ ^b	79	75–85
			VCI	109	103–114
			WMI	80	74–89
			PRI	72	66–81
			PSI	59	54–73
7.2	18	WAIS-IV	FSIQ ^b	96	91–101
			VCI	113	107–118
			WMI	92	85–100
			PRI	100	92–108
			PSI	76	69–88
8.3	50	WAIS-IV short form	VCI	93	88–99
10.4 ^c	26	WAIS-III	FSIQ ^b	68	65–73
			VCI	79	74–85
			WMI	68	63–77
			PRI	61	56–72
			PSI	55	51–69
11.5 ^c	42	WAIS-IV	FSIQ	50	47–55
			VCI	66	62–73
			WMI	55	51–64
			PRI	52	48–61
			PSI	50	47–63
Cases from literature
1 [17]	31	WAIS	FSIQ	88	NK
2 [17]	36	WAIS	FSIQ	75	NK

Neurocognitive test scores are based on a mean of 100 with a SD of 15.
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.
^a VCI and PRI estimated using the proration method [25], extrapolating the scores on 2 subtest within each index.
^b FSIQ should be interpreted with caution given significant discrepancies between the index scores for the different components.
^c Functions with a mild neurocognitive impairment.

ISSN: 1750-1172