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Journal of Child Neurology, Vol. 18, No. 5, 348-355 (2003)
DOI: 10.1177/08830738030180050301

Ocular Motor Behavior of Children With Neurofibromatosis 1

Adrian G. Lasker, MD

Department of Neurology, Johns Hopkins School of Medicine, Baltimore, alasker{at}dizzy.med.jhu.edu

Martha B. Denckla, MD

Department of Neurology, Johns Hopkins School of Medicine, Departments of Psychiatry and Pediatrics Johns Hopkins School of Medicine, Kennedy Krieger Institute Baltimore, MD

David S. Zee, MD

Department of Neurology, Johns Hopkins School of Medicine

Patients with neurofibromatosis 1 show neurocognitive deficits including abnormal visuospatial performance, but oculomotor behavior has not been studied. We recorded saccades in neurofibromatosis 1 and normal children, ages 6 to 12 years. Patients showed increased latency and diminished amplitude to more eccentric targets for both visually guided and memory-guided saccades. Subtracting the latencies of visually guided saccades from memory-guided saccades produced no differences between groups. For predictive saccades (0.5 Hz), patients with neurofibromatosis 1 generated fewer anticipatory saccades than normal subjects, but patients with neurofibromatosis 1 made more direction errors in an antisaccade task. Patients with neurofibromatosis 1 show saccade abnormalities that are not easily ascribed to dysfunction within single cortical areas. Rather, the findings likely reflect (1) partial involvement of different components of a widely distributed cerebral network that controls saccades, (2) impaired visuomotor maturation in a brain abnormal early in development, and (3) excessive {gamma}-aminobutyric acid—mediated inhibition, which could influence basal ganglia pathways that modulate superior colliculus activity. (J Child Neurol 2003;18:348—355).


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