BACKGROUND: In patients with neurofibromatosis type 1 (NF1), cognitive deficits are frequent manifestations. They are associated with focal areas of high signal intensity (T2H) on T2-weighted MRI of the brain. Changes in T2H may affect cognitive development. Our study was to analyse the relations between the long-term development of T2H development and the cognitive abilities in patients with NF1., METHOD: In a controlled prospective study, we investigated 67 patients with NF1 for their IQ with the patients having T2H (MRI) examination. Assessments and MRI were repeated at a 3- year follow-up., RESULTS: Patients without T2H performed at the average IQ level. Patients with stable T2H performed below average level but within normal limits of IQ. Patients with T2H that decreased over study period performed well below normal limits at first examination but within limits at follow-up. Stable T2H were found primarily in the cerebellum and the capsula interna. T2H that decreased were found primarily in the thalamus and the basal ganglia., CONCLUSIONS: T2H in the cerebellum and the capsula interna are more permanent but exert a minor left shift in IQ. T2H in the thalamus or the basal ganglia are related to severely reduced performance. Decreasing they give way for performance improvement. There may be a different pathology in T2H related to the intracranial regions the T2H affect in patients with NF1.Copyright © 2010 The Author(s)/Journal Compilation © 2010 Foundation Acta Paediatrica.
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