关键词: Ⅰ型神经纤维瘤病; 烟雾综合征; 临床表现

Abstract:

【Abstract】 Objective The aim of this study was to discuss the clinical features, imaging characteristics and prognosis of neurofibromatosis type I (NFI)-moyamoya syndrome (MMS). Methods Four consecutive patients with NFI-MMS between January 2003 and March 2012 were obtained retrospectively from 307 Hospital of Chinese People's Liberation Army. The data included the symptoms onset, age, Suzuki stages, treatment and symptomatic progression. Results The ratio of male-to-female patients was 1∶3, the age onset was under the age of 18 in 3 cases and another case was 23 years old. For the onset of symptoms, 2 cases presented with transient ischemic attack (TIA), the other 2 cases appeared headache and cerebral infarction. For Suzuki's stage, in 6 sides of 4 patients, there were 2 sides in stage 1, 1 side in stage 2, 2 sides in stage 4 and 1 side in stage 6. Three patients received revascularization surgery, the average follow-up time was from 10 to 72 months, and the symptoms improved significantly during the follow-up. Conclusion MMS associated with NFI occurred more commonly in childhood, the most common symptoms was ischemia, and the degree of vascular stenosis can be presented with each Suzuki's stage. Revascularization surgery may improve the clinical symptoms and the prognosis of patients.

Key words: Neurofibromatosis type I; Moyamoya syndrome; Clinical features