Abstract
154 Objectives About 65% of NF1 children perform below chronologic age in school and 45% of them require special education assistance between the ages of 8 and 16 years. The cognitive phenotype of NF1 pediatric patients is not well understood. We retrospectively evaluated the cerebral FDG PET of NF1 pediatric patients using voxel-based statistical analysis. Methods Six NF1 patients (5 male, 1 female median age 13) underwent whole-body FDG PET/CT (Gemini TF, Philips Healthcare) including the brain, for evaluation of extra-cranial neoplasm. Their brain PET images were extracted from the whole-body images and compared with those of a comparison set consisting of 21 pediatric patients using Statistical Parametric Mapping SPM8 software (Wellcome Department of Cognitive Neurology, University College London, UK). The comparison set subjects underwent PET/CT for extracranial neoplasm not related to NF1, had no history of brain tumor or neurolopsychologic disease and had not received chemotherapy prior to PET/CT. SPM parameters included a 6 mm smoothing kernel, proportional scaling of global counts and a two-sample t test. Significant differences between groups were examined at p Results Compared with the comparison set, the 6 NF1 pediatric patients showed large (> 500 voxels) symmetric hypometabolic clusters in both thalami and caudate nuclei as well as large asymmetric clusters in the right medial frontal lobe and left occipital lobe. At smaller clusters (100-500 voxels), additional hypometabolic clusters were present in bilateral temporal lobe along the posterior horn, right greater than left, suggestive of the optic radiation pathway. Conclusions The striking FDG reductions of the bilateral thalamus and caudate nucleus in children is consistent with previous studies. The reduced metabolism in the occipital lobe and along the presumed bilateral optic radiations may explain the visuospacial deficits typically seen in NF1 children
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