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本文报告了９例神经纤维瘤病颅脑的影像表现（重点Ｘ线、ＣＴ），其中８例经手术及病理证实，１例局部活检证实。全部病例均有皮肤多发性结节、色素斑及智力低下，部分病例有神经性耳聋、视力障碍及遗传史等。颅脑影像特征：①颅面骨异常（８例），包括颅盖骨缺损、颅板变簿，内听道、蝶鞍、眼眶、视神经孔扩大和骨质缺损。②颅内肿瘤：双侧听神经病（５例）、脑膜瘤（４例）、神经交质瘤（１例）。青年人患双侧听神经瘤并发脑膜瘤或视神经瘤，尤其多发性脑膜瘤可为诊断本病的重要依据。本文就本病诊断标准、颅脑影像的临床意义及鉴别诊断进行了讨论。
This article reports the imaging features of 9 cases of neurofibromatosis brain (focus X-ray, CT), of which 8 cases were confirmed by surgery and pathology, 1 case confirmed by local biopsy. All cases had multiple skin nodules, pigmented spots, and mental retardation. Some cases had neurological deafness, visual impairment, and genetic history. Brain imaging features: 1 craniofacial bone abnormalities (8 cases), including calvarial defect, skull plate book, inner auditory canal, sella, orbit, optic nerve hole enlargement and bone defect. 2 Intracranial tumors: bilateral auditory neuropathy (5 cases), meningioma (4 cases), neuroglioma (1 case). Young people suffering from bilateral acoustic neuroma with meningioma or optic neuroma, especially multiple meningioma can be an important basis for the diagnosis of this disease. This article discusses the diagnostic criteria of this disease, the clinical significance of craniocerebral imaging, and differential diagnosis.