Franck G, Sadzot B, Salmon E, Reznik M, van der Linden M, Schmidt S, Maquet P
Université de Liège. Clinique neurologique, Belgique.
Rev Neurol (Paris). 1987;143(10):657-69.
We report a case of limbic encephalopathy clinically characterized by a progressive amnestic syndrome and many EEG seizures mainly localized on the left temporal area. Biological investigations revealed diabetes mellitus and a syndrome of inappropriate antidiuretic hormone secretion (IADH). Haemodynamic and metabolic studies by positron-emission tomography showed an important increase in cerebral blood flow (CBF) and cerebral metabolic rate of oxygen on the left anterior temporal region precisely where the electrical seizures were recorded. Nine months later, severe disorders of memory and a dramatic decrease in CBF and CMRO2 on the same area region were present. At autopsy, a small size oat cell bronchial carcinoma was found with metastases in two small adjacent lymph nodes. Neuropathological examination showed atrophy (neuronal loss, protoplasmic gliosis) in the amygdala; where there was in addition an area of nodular gliosis. The hippocampus and parahippocampal gyrus lesions were severe on the left and moderate on the right side. The authors discuss the nosology of their case in the paraneoplastic syndromes and, with a review of the literature, the role of ADH and cellular hyperactivity in the pathogenesis of specifically localized neuronal alterations.
我们报告一例边缘叶性脑病,临床表现为进行性遗忘综合征,脑电图显示多次癫痫发作,主要定位于左侧颞区。生物学检查发现糖尿病和抗利尿激素分泌不当综合征(SIADH)。正电子发射断层扫描的血流动力学和代谢研究显示,左侧前颞区脑血流量(CBF)和脑氧代谢率显著增加,而该区域正是记录到癫痫放电的部位。九个月后,同一区域出现严重的记忆障碍,CBF和CMRO2显著下降。尸检发现一个小的燕麦细胞支气管癌,并伴有两个相邻小淋巴结转移。神经病理学检查显示杏仁核萎缩(神经元丢失、原浆性胶质细胞增生),此外还有一个结节性胶质细胞增生区域。海马和海马旁回病变左侧严重,右侧中度。作者讨论了该病例在副肿瘤综合征中的分类,并通过文献复习,探讨了抗利尿激素和细胞功能亢进在特定部位神经元改变发病机制中的作用。