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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.)
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