Article

Motor skill impairment and cognitive dysfunction are commonly reported
features of neurofibromatosis type 1 (NF1). We characterized and determined the
relationship between motor impairment, gait variables, and cognitive function in
children and adolescents with NF1. METHOD: Motor function, gait, and
neurocognitive abilities were assessed in 46 children and adolescents with NF1
(26 males, 20 females; age range 7-17 y; mean age 11 y 1 mo, SD 3 y 2 mo). Tests
to establish correlations between neurocognitive, motor, and gait variables were
performed. RESULTS: Compared with normative data, 28/39 of our NF1 cohort
demonstrated impaired performance for balance and upper limb coordination and
16/38 for running speed and agility. Gait data revealed a strategy to preserve
balance at the expense of velocity, with the unexpected exception of a tendency
for reduced base of support. Neurocognitive testing confirmed mean IQ in the low
average range (86.0) and deficits in spatial working memory and strategy
generation. Significant correlations between a number of neurocognitive measures
and motor abilities and gait were identified. The largest associations were
between gait width and spatial working memory (r=0.594) and running speed and
agility with strategy generation (r=0.549). INTERPRETATION: We have identified a
relationship between balance, running speed and agility, gait, and cognition in
children with NF1. Findings suggest a shared abnormal neurodevelopmental process
underlying some cognitive and motor abilities in NF1. Results are discussed
within the context of evidence highlighting abnormal dopamine-mediated
corticostriatal circuitry in NF1.
CI - (c) 2014 Mac Keith Press.

Langue : ANGLAIS