Wang Xianling, Yang Yanhui, Wang Xiangbo, Li Cunjiang
Neurol Res. 2014 Feb;36(2):137-42. doi: 10.1179/1743132813Y.0000000273. Epub 2013 Dec 6.
The objective of this study was to improve the knowledge of the clinical and MRI presentation of general paresis of the insane (GPI) and achieve its early diagnosis. Fifteen patients with GPI were gathered. Their clinical data and brain MRI information were analyzed. The age range was 33-62 years (mean: 48·8 years). The disease lasted 34 days-28 months (mean: 10·87 months). Treponema pallidum hemagglutination assays (TPHAs) on the cerebrospinal fluid (CSF) and sera were positive. All patients were human immunodeficiency virus (HIV) negative and primarily showed progressive cognitive impairment, mostly accompanied with emotional disturbance and psychosis. The accompanying symptoms included epilepsy (n = 7), ataxia (n = 5), cerebral vascular disease (n = 3), and limbs tremor (n = 2). Typical Argyll Robertson's pupil was observed in two cases. A CSF examination showed elevated white blood cell (WBC) and protein level. Purely cerebral atrophy was found in three cases. Abnormal high signals were found in nine cases in different brain area, mainly in bilateral temporal, insular, and frontal lobes and hippocampus in T2-weighted and fluid-attenuated inversion recovery (FLAIR), which was accompanied by cerebral and/or hippocampus atrophy. Purely atrophy of bilateral or unilateral hippocampus was found in two cases. Cerebral infarction lesions were found in three cases. Contrary to the previous reports, the hippocampal atrophy and bilateral abnormal signals of hippocampus and temporal lobe in brain MRI were more common in cases with GPI. Abnormal signals were probably related to proliferation of glial cells rather than cytotoxic edema. The pathogenesis of the abnormal signals needs to be further explored. General paresis of the insane has a wide variety of central nervous system manifestations, either clinically or neuroradiologically. Identification of these presentations could be important for early diagnosis.
本研究的目的是提高对麻痹性痴呆(GPI)临床及MRI表现的认识并实现早期诊断。收集了15例GPI患者,分析其临床资料及脑部MRI信息。年龄范围为33 - 62岁(平均48.8岁)。病程为34天至28个月(平均10.87个月)。脑脊液(CSF)和血清的梅毒螺旋体血凝试验(TPHA)均为阳性。所有患者人类免疫缺陷病毒(HIV)阴性,主要表现为进行性认知障碍,多伴有情绪障碍和精神病。伴随症状包括癫痫(7例)、共济失调(5例)、脑血管病(3例)和肢体震颤(2例)。2例观察到典型的阿-罗瞳孔。脑脊液检查显示白细胞(WBC)和蛋白水平升高。3例发现单纯性脑萎缩。9例在不同脑区发现异常高信号,主要在T2加权像和液体衰减反转恢复(FLAIR)序列的双侧颞叶、岛叶、额叶及海马,同时伴有脑和/或海马萎缩。2例发现双侧或单侧海马单纯性萎缩。3例发现脑梗死病灶。与既往报道不同,GPI患者脑部MRI表现为海马萎缩及海马和颞叶双侧异常信号更为常见。异常信号可能与胶质细胞增生而非细胞毒性水肿有关。异常信号的发病机制有待进一步探讨。麻痹性痴呆在临床及神经放射学方面均有多种中枢神经系统表现。识别这些表现对早期诊断可能很重要。