摘要
目的系统的总结、复习神经皮肤综合征的影像学特征和诊断。方法收集36例经临床和手术证实的此类病例,对其影像学资料进行回顾性的总结和分析。结果①结节性硬化17例,12例CT平扫见两侧脑室室管膜下多发小结节状高密度钙化灶,3例在幕上脑实质内有斑点状钙化,2例见等或稍高密度的结节突入脑室内和脑室周围,并发右侧脑室内星形细胞瘤1例;②神经纤维瘤病7例,影像学表现为颅骨和椎管异常,椎体后缘呈明显的切凹改变;③脑颜面血管瘤综合征6例,X线平片和CT可见顶枕部沿脑回分布的弯曲的条状高密度钙化,部分延伸致侧脑室内,增强后见病灶内有扭曲的条状和结节状明显强化的血管影;④视网膜小脑血管瘤病6例,影像学表现为小脑大囊、小结节样占位性病变3例,1例伴有出血改变,1例伴发视网膜血管瘤,1例胸段髓内病变合并全脊髓广泛的空洞形成;病灶MRI呈等T1WI和稍高的不均匀T2WI信号,注射Gd-DTPA后病灶呈明显的均匀性强化。结论结合典型的临床症状和体征,合理应用影像学检查手段,分析不同的影像学征像,可以对神经皮肤综合征做出明确的诊断。
Objective To summarize the imaging features and diagnosis of neurocutaneous syndrome. Methods Imaging data of 36 patients with neurocutaneous syndrome, proved by operation and clinical symptoms, were retrospectively analyzed. Results ① Of all 17 cases of tuberous sclerosis complex （TSC）, subependymal nodular calcification lesions were found in 12 cases on precontrast CT image, patchy calcification lesions in brain were found in 3 cases, iso- or slight hyper-density nodules protruded into ventricle and around ventricle were found in 2 cases, and accompanied astrocytoma was found in 1 case. After using contrast media, nodules without calcification and astrocytoma were moderately enhanced. These nodules appeared as isodensity signal on precontrast T1WI, were markedly enhanced on postcontrast MRI. ② All 7 cases of neurofibromatosis in the report displayed abnormal skull and spinal canal, concave change of vertebral. Enlarged dura spinal sac were found on CT and MRI, partly stretching to nerve root. Three cases of bilateral vestibular schwannomas, 2 of optic nerve glioma, and 1 meningioma were found. ③ Gyriform cortical calcification lesions were found in 6 patients with Sturge-Weber syndrome on plain film of skull and CT image of brain. In these lesions, notorious strip, nodular and markedly enhanced vessels were also found after intravenous shot of contrast agents. ④ A- mong 6 cases of von Hipple Linda, 3 cases appeared as large cyst with mural nodule in cerebelum, 2 cases as heterogeneous density solid mass in supratentor. Mural nodule and solid mass had marked enhancement on enhanced CT image. The other case showed iso-density signal on T1WI, heterogeneous hyper-density signal on T2WI and marked enhancement on post-contrast T1WI in spinal cord. Of the 6 cases, one case accompanied with hemorrhage, l with retinal angioma, 3 with multi cyst of liver and kidney, I with syringomyelia. Conclusion Diagnosis of neurocutaneous syndrome can be clearly made by using optimal imaging examination, analyzing all kinds of imaging features and associating typical clinical syndrome.
出处
《第三军医大学学报》
CAS
CSCD
北大核心
2008年第14期1381-1384,共4页
Journal of Third Military Medical University
