Duffner P K, Cohen M E, Seidel F G, Shucard D W
Department of Neurology, State University of New York, School of Medicine, Buffalo 14222.
Neurology. 1989 Mar;39(3):373-8. doi: 10.1212/wnl.39.3.373.
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.
我们对47名患有神经纤维瘤病的儿童进行了前瞻性评估，以确定先前报道的磁共振成像（MRI）上的高信号（T2延长）是否与CT、脑干听觉诱发电位（BAER）、脑电图（EEG）、临床检查、认知能力或癫痫症相关。30%的儿童有癫痫病史，70%有学习障碍或智力迟钝。总体而言，74%的儿童MRI检查异常。62%在T2加权图像上有高信号（T2延长）。异常信号主要位于基底神经节、脑干和小脑。25%的患者脑电图异常，28%的患者CT异常，27%的患者BAER检查异常。MRI上的异常信号与CT、BAER、脑电图、学校安置或临床检查结果并不一致相关。异常信号可能反映了脑实质异常区域，要么是错构瘤、异位，要么是局部脑发育异常区域。
