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Cognitive deficits in neurofibromatosis Type 1 (NF-1) have
been documented in both the verbal and visuospatial domains.
Previous investigations from our laboratory have determined
a specific pattern of “spared” (Picture Arrangement,
Picture Completion, and Rapid Automatized Naming) and
“impaired” (Judgment of Line Orientation, Vocabulary,
and Block Design) performance on cognitive measures in this
population when compared to sibling-matched controls in pairwise
designs. Growth curve analyses were conducted on these repeated
measures in 19 patients with NF-1 and their siblings to investigate
the longitudinal course and growth pattern of these spared and
impaired measures. Results indicated that over time children
with NF-1 do not catch up to their siblings on impaired measures,
and they continue to perform similarly to their siblings on
the spared measures. With respect to growth rates, on average
across the 6 cognitive measures there was no significant difference
between the groups. However, the variation among families
for level of performance was estimated to be larger than variation
among siblings within a family for 2 out of 6 cognitive measures
(i.e., providing for these 2, Vocabulary and Rapid Automatized
Naming, evidence of substantial familial correlation), suggesting
that there is need to consider NF-1 associated deficits within
a familial context. (JINS, 2002, 8, 838–846.)
This study assessed two relevant aspects of executive
dysfunction in children with either Tourette syndrome (TS)
or ADHD. Process variables derived from existing neuropsychological
measures were used to clarify the executive function construct.
Clustering of responses on measures of verbal fluency,
figural fluency, and verbal learning was examined to assess
strategic response organization. Rule breaks, intrusions,
and repetition errors were recorded to assess inhibition
errors. No significant differences were found among the
three groups (TS, ADHD, and controls) on tasks of response
organization (clustering). In our sample, both the ADHD
and the TS groups were largely free from executive function
impairment, and their performance on the fluency and list
learning tasks was in the average range. There was a significant
group difference on one of the disinhibition variables,
with both TS and ADHD groups showing significantly more
intrusions on verbal list learning trials than controls.
When more traditional total score variables were analyzed
among the three groups, there were no significant differences;
however, analysis of effect size revealed medium-to-large
effect sizes for Letter Word Fluency total score differences
(ADHD vs. controls), and for Semantic Word Fluency
total score differences (ADHD vs. TS), with the
ADHD group having weaker performance in both comparisons.
Results provide some support for the use and analysis of
process variables—particularly those related to inhibition
and intrusion errors, in addition to the total score variables
when assessing executive function deficits in children
with ADHD and TS. While group differences may be found,
children with uncomplicated TS should not routinely be
considered to have significant executive function impairments,
and when deficits are found, they may be attributable to
other comorbid disorders. (JINS, 2001, 7,
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