Our study indicates that individuals with TI deletion generally have more behavioral and psychological problems than individuals with the TII deletion or UPD. Four recently identified genes have been identified in the chromosome region between BP1 and BP2 with 1 of the genes (NIPA-1) expressed in mouse brain tissue but not thought to be imprinted. It may be important for brain development or function. These genes are deleted in individuals with TI deletion and are implicated in compulsive behavior and lower intellectual ability in individuals with TI versus TII.
Purpose
The mean length of children’s utterances is a valuable estimate of their early language acquisition. The available normative data lacks documentation of language and nonverbal intelligence levels of the samples. This study reports age-referenced MLU data from children with specific language impairment and children without language impairments.
Method
306 child participants were drawn from a data archive, ages 2;6–9;0 years, 170 with SLI and 136 control children. 1564 spontaneous language samples were collected, transcribed and analyzed for sample size and MLU in words and morphemes. Means, standard deviations, and effect sizes for group differences are reported for MLUs, along with concurrent language and nonverbal intelligence assessments, per 6-month intervals.
Results
The results document an age progression in MLU words and morphemes, and a persistent lower level of performance for children with SLI.
Conclusions
The results support the reliability and validity of MLU as an index of normative language acquisition and a marker of language impairment. The findings can be used for clinical benchmarking of deficits and language intervention outcomes, as well as comparisons across research samples.
Advances in genetics have led to an increased understanding of the role of the genotype on behavioural functioning. The purpose of the present study was to examine differences in intellectual functioning in individuals with Prader-Willi syndrome (PWS) with a paternal 15q11-q13 deletion versus maternal uniparental disomy (UPD) of chromosome 15. Measures of intelligence and academic achievement were administered to 38 individuals with PWS (24 with deletion and 14 with UPD). The subjects with UPD had significantly higher verbal IQ scores than those with deletion (P< 0.01). The magnitude of the difference in verbal IQ was 9.1 points (69.9 versus 60.8 for UPD and deletion PWS subjects, respectively). Only 17% of subjects with the 15q11-q13 deletion had a verbal IQ > or = 70, while 50% of those with UPD had a verbal IQ > or = 70. Performance IQ scores did not differ between the two PWS genetic subtype groups. This is the first report to document the difference between verbal and performance IQ score patterns among subjects with PWS of the deletion versus the UPD subtype.
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