神经纤维瘤病是一种遗传性错构瘤性疾病,可侵全身各器官和系统,其中头颅改变占40％。本病颅脑影像学表现具有一定特征性,可以诊断或揭示本病的存在。近十年来,我院收治6例,其中5例经手术病理证实,1例局部活检证实,报道如下: 临床资料男女各3例,年龄:18～30岁,平均23岁。临床表现:双耳听力障碍5例;走路不稳3例;眶周围肿物或突眼3例;颅内压增高2 Neurofibromatosis is a hereditary hamartoma that can invade all organs and systems of the body, with head changes accounting for 40%. The brain imaging performance of this disease has a certain characteristic, you can diagnose or reveal the existence of the disease. In the past ten years, 6 cases were admitted to our hospital, of which 5 cases were confirmed by surgery and pathology, and 1 case was confirmed by local biopsy. The report is as follows: Clinical data of 3 males and 3 females, aged from 18 to 30 years with an average of 23 years old. Clinical manifestations: 5 cases of binaural hearing impairment; walking instability in 3 cases; 3 cases of orbital tumors or exophthalmos; increased intracranial pressure 2