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Departments of Neurology and Pediatrics, State University of New York at Buffalo, School of Medicine, Buffalo, NY. (Drs. Duffner, Cohen, and Shucard)
Department of Radiology, State University of New York at Buffalo, School of Medicine, Buffalo, NY. (Dr. Seidel)
We prospectively evaluated 47 children with neurofibromatosis to determine whether the previously reported high signals on magnetic resonance imaging (MRI) (prolonged T2) correlated with CT, brainstem auditory evoked responses (BAER), EEG, clinical examinations, cognitive abilities, or seizure disorder. Thirty percent of children had a history of seizures and 70% had either learning disabilities or mental retardation. Overall, 74% had an abnormal MRI examination. Sixty-two percent had high signals (prolonged T2) on T2-weighted images. Abnormal signals were located primarily in the basal ganglia, brainstem, and cerebellum. Twenty-five percent of patients had abnormal EEGs, 28% had abnormal CTs, and 27% had abnormal BAER examinations. The abnormal signals on MRI did not consistently relate to findings on CT, BAER, EEG, school placement, or clinical examination. The abnormal signals presumably reflect areas of abnormal brain parenchyma, either hamartomas, heterotopias, or local areas of brain dysplasia.
Address correspondence and reprint requests to Dr. Duffner, Department of Neurology and Pediatrics, State University of New York at Buffalo, School of Medicine, 219 Bryant Street, Buffalo, NY 14222.
Received May 31, 1988. Accepted for publication in final form September 26, 1988.
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