Kawauchi M, Gibo H, Kobayashi S, Sugita K
Department of Neurosurgery, Shinshu University Faculty of Medicine, Matsumoto, Japan.
No Shinkei Geka. 1988 Feb;16(2):189-94.
We present two cases of Werner's syndrome associated with intracranial meningioma. Characteristic clinical features of Werner's syndrome include short stature with slender extremities, premature senility, juvenile cataract, skin changes, a tendency to diabetes mellitus and familial occurrence. A 44-year-old female, who had been treated for diabetes mellitus, was diagnosed as having Werner's syndrome because of various characteristic features. A falx meningioma was incidentally found on CT scan, and was surgically removed. Her diabetes mellitus improved. The second case was a 28-year-old male was diagnosed as having Werner's syndrome, diabetes mellitus, juvenile cataract, together with diabetes insipidus, and liver dysfunction. He developed severe headache, gait disturbance and then became unconscious with right hemiparesis. He was found to have a parasagittal meningioma by CT scan and angiography. After removal of the tumor, diabetes mellitus, diabetes insipidus and liver dysfunction improved. The reported incidence of neoplasms associated with Werner's syndrome is about 10%. The majority of associated tumors were mesenchymal in origin. Ten meningiomas, 1 neurinoma and 2 gliomas are reported as associated tumors in the central nervous system. Most of the associated meningiomas were asymptomatic and found incidentally at autopsies or CT scans. Diabetes mellitus associated with Werner's syndrome is generally mild with high immunoreactive insulin value and is controllable by diet therapy and oral antidiabetic drugs. Daily profile of blood sugar improved after the removal of tumor in our cases. In 50 gm glucose tolerance test, tendency of delayed appearance of peak value, which is common in Werner's syndrome, was not altered in our cases. Discussion is made as to the association of Werner's syndrome with meningioma and diabetes mellitus.
我们报告两例与颅内脑膜瘤相关的沃纳综合征病例。沃纳综合征的典型临床特征包括身材矮小且四肢细长、早衰、青少年白内障、皮肤改变、患糖尿病的倾向以及家族性发病。一名44岁的女性，曾因糖尿病接受治疗，因具有各种典型特征而被诊断为患有沃纳综合征。CT扫描偶然发现大脑镰脑膜瘤，并进行了手术切除。她的糖尿病病情有所改善。第二例是一名28岁男性，被诊断为患有沃纳综合征、糖尿病、青少年白内障，同时还有尿崩症和肝功能障碍。他出现严重头痛、步态障碍，随后昏迷并伴有右侧偏瘫。通过CT扫描和血管造影发现他患有矢状窦旁脑膜瘤。肿瘤切除后，糖尿病、尿崩症和肝功能障碍均有所改善。据报道，与沃纳综合征相关的肿瘤发病率约为10%。大多数相关肿瘤起源于间叶组织。据报道，中枢神经系统中有10例脑膜瘤、1例神经鞘瘤和2例胶质瘤为相关肿瘤。大多数相关脑膜瘤无症状，是在尸检或CT扫描时偶然发现的。与沃纳综合征相关的糖尿病一般较轻，免疫反应性胰岛素值较高，可通过饮食疗法和口服降糖药控制。在我们的病例中，肿瘤切除后血糖的日常情况有所改善。在50克葡萄糖耐量试验中，我们病例中沃纳综合征常见的峰值出现延迟的趋势并未改变。本文对沃纳综合征与脑膜瘤及糖尿病的关联进行了讨论。
