Acute-Onset Cerebellar Symptoms in Lhermitte–Duclos Disease: Case Report

Adult-onset Lhermitte–Duclos disease (LD), or dysplastic cerebellar gangliocytoma, is a hamartoma considered pathognomonic for Cowden disease. Classically, LD has a progressive and insidious onset of symptoms. In this case report, we present a patient having rapid neurological deterioration from acute-onset LD. There are only three reported cases of acute LD presentation. A 22-year-old female presented to the emergency department with diplopia, dysarthria, dysphagia, and gait instability which developed within 6 h. A non-contrast CT scan revealed diffuse attenuation in the left cerebellum and mild ventricular dilatation. LP revealed no organisms. Magnetic resonance imaging revealed salient “tiger stripe” appearance of the left cerebellar cortex and effacement of the fourth ventricle. The patient subsequently underwent suboccipital craniotomy and gross total resection of the lesion. The tumor histology showed distortion of normal cerebellar architecture with dysplastic ganglion cells, loss of Purkinje cells, atrophy of the white matter, and expansion of cerebellar folia. Findings were consistent with adult-onset Lhermitte–Duclos disease.     

覆膜支架在颈内动脉海绵窦段病变治疗中的应用

目的 评价应用覆膜支架治疗颈内动脉海绵窦段病变的价值.方法 采用覆膜支架治疗11例颈内动脉海绵窦段病变,其中颈内动脉海绵窦瘘5例,颈内动脉海绵窦段动脉瘤6例,术后1年行全脑血管造影随访和临床随访.结果 11例患者中,成功应用覆膜支架治疗9例,成功置入覆膜支架的9例患者,术后即刻血管造影显示病变完全消失,临床症状逐渐好转,无手术相关并发症,术后1年行全脑血管造影复查8例,结果显示病变消失,责任动脉均保持通畅.结论 覆膜支架在处理颈内动脉海绵窦段动脉瘤或颈内动脉海绵窦瘘方面,有治疗成功率高,疗效好,并发症少,复发率低等特点,值得推广.     

脑脊液CFP-10和Ag85检测对结核性脑膜炎的诊断价值

研究背景结核性脑膜炎的早期诊断目前仍是临床难点,寻找结核性脑膜炎早期特异性诊断指标是目前研究热点。本文主要探讨脑脊液培养分泌蛋白10(CFP10)和Ag85蛋白复合物表达水平对结核性脑膜炎的临床诊断价值。方法采用酶联免疫吸附试验分别检测结核性脑膜炎(30例)、非结核性颅内感染(27例)和对照(29例)受试者脑脊液CFP10和Ag85蛋白复合物表达水平。结果结核性脑膜炎组患者脑脊液CFP10和Ag85中位表达水平分别为0.74和1.10pg/ml,均高于非结核性颅内感染组[(0.02±0.01)、(0.54±0.10)pg/ml]和对照组[(0.02±0.01)、(0.52±0.11)pg/ml],组间差异有统计学意义(H=60.958,P=0.000;H=57.972,P=0.000)。非结核性颅内感染组与对照组之间差异无统计学意义(t=1.128,P=0.253;t=0.980,P=0.329)。结论结核性脑膜炎患者脑脊液CFP10和Ag85蛋白复合物表达水平高于非结核性颅内感染患者和对照者,检测这两项实验室指标的变化可协助结核性脑膜炎的早期诊断。     

Estimated low risk of rupture of small-sized unruptured intracranial aneurysms (UIAs) in relation to intracranial aneurysms in patients with subarachnoid haemorrhage

Objective

International guidelines for the management of unruptured intracranial aneurysms (UIAs) recommend observation in aneurysms <10 mm due to the estimated low risk of rupture. The aim of our study was analyse the data of recently treated patients with ruptured cerebral aneurysms with the special focus on size and configuration in view of the frequency scale in a daily routine setting.

Methods

We reviewed the data of all patients with aneurysmal subarachnoid haemorrhage (SAH) during the last 24 months at our institution. Configuration and size of the aneurysms were measured. Clinical data were collected using the following classifications for analysis: Hunt and Hess (H&H), modified Rankin Scale (mRS) and Fisher classification.

Results

Data of 135 patients with aneurysmal SAH (98 women, 37 men; ratio 2.6:1) were analysed. Analysis showed that 19 aneurysms (14 %) were >10 mm (mean size, 19.2 mm) and 116 aneurysms (85.9 %) <10 mm (mean size, 6.2 mm). In total, 112 were categorised as berry-like configured aneurysms (n?=?113 <10 mm, n?=?3 >10 mm), 18 as multi-lobar (n?=?16 <10 mm, n?=?2 >10 mm) and 5 as fusiform (n?=?4 <10 mm, n?=?1, >10 mm).

Conclusion

Since the results of our study showed that the majority of the aneurysms are <10 mm (mean, 6.2 mm), it is justified to challenge the recommendations of the international guidelines in a daily routine setting. We believe that the published data are not convincing enough to play a guidance role in daily routine. Due to improving surgical and endovascular techniques with satisfying results and the high number of ruptured small aneurysms, we believe a change in attitude in management of small-sized aneurysms is needed. Further diagnostic models are needed to determine the risk of rupture of intracranial aneurysms properly to obtain adequate treatment for UIAs.     

Retrospective review of rectal cancer surgery in northern Alberta

Introduction

Previous studies, including research published more than 10 years ago in Northern Alberta, have demonstrated improved outcomes with increased surgical volume and subspecialisation in the treatment of rectal cancer. We sought to examine contemporary rectal cancer care in the same region to determine whether practice patterns have changed and whether outcomes have improved.

Methods

We reviewed the charts of all patients with rectal adenocarcinoma diagnosed between 1998 and 2003 who had a potentially curative resection. The main outcomes examined were 5-year local recurrence (LR) and disease-specific survival (DSS). Surgeons were classified into 3 groups according to training and volume, and we compared outcome measures among them. We also compared our results to those of the previous study from our region.

Results

We included 433 cases in the study. Subspecialty-trained colorectal surgeons performed 35% of all surgeries in our study compared to 16% in the previous study. The overall 5-year LR rate and DSS in our study were improved compared to the previous study. On multivariate analysis, the only factor associated with increased 5-year LR was presence of obstruction, and the factors associated with decreased 5-year DSS were high-volume noncolorectal surgeons, presence of obstruction and increased stage.

Conclusion

Over the past 10 years, the long-term outcomes of treatment for rectal cancer have improved. We found that surgical subspecialization was associated with improved DSS but not LR. Increased surgical volume was not associated with LR or DSS.     

氟对大鼠甲状腺过氧化物酶mRNA表达的影响

目的 观察长期氟过量对大鼠甲状腺过氧化物酶(TPO)活性、TPO mRNA表达的影响,并探讨其可能的机制.方法 选用雄性SD大鼠40只,按体质量分为对照组、低氟组、中氟组、高氟组,每组10只,分别饮用含氟化钠0.40(普通自来水)、15.00、30.00和60.00 mg/L的自来水,均食用普通粮食配制的饲料.喂养180 d后处死.测定血清FT3和FT4;采用改良愈创木酚法测定甲状腺TPO活性;半定量RT-PCR方法检测甲状腺TPO mRNA表达水平.结果 低、中、高氟组血清FL3水平[(3.62±0.47)、(3.57±0.55)和(3.30±0.68)pmol/L]与对照组[(3.64±0.45)pmol/L]相比虽呈下降趋势,但差异无统计学意义(P＞0.05);高氟组血清FT4水平[(8.64±1.72)pmol/L]低于对照组、低、中氟组[(13.08±1.69)、(12.68±1.32)、(12.05±1.43)pmol/L,P均＜0.05].对照组、低氟组、中氟组、高氟组甲状腺细胞TPO活性[(1.572±0.064)、(1.414±0.086)、(1.322±0.049)、0.960±0.083)U/L]随着氟浓度的升高而明显降低,任意两组组间比较差异均有统计学意义(P均＜ 0.05),TPO活性与氟浓度呈显著负相关(r=-0.955,P＜0.05).半定量RT-PCR结果显示:对照组、低氟组、中氟组、高氟组甲状腺TPO mRNA表达水平(0.936±0.160、0.368±0.095、0.115±0.018、0.016±0.008)随着氟浓度升高而明显降低,任意两组组间比较差异均有统计学意义(P均＜0.05).结论 长期摄入过量氟抑制甲状腺TPO活性和TPO的表达,影响甲状腺激素合成.     

血管成形术后血管外膜改变与血管重塑的相关性研究

目的:探讨大鼠胸主动脉血管成形术后血管外膜激活与血管重塑的相关性。方法:用6F人冠状动脉快速交换球囊损伤大鼠胸主动脉,术后2周和6周取材,行血管形态学定量分析,并行增殖细胞核抗原(PCNA)免疫组织化学染色,观察PCNA在血管外膜上的表达。结果:血管成形术组血管外膜细胞数量、血管外膜细胞增殖指数,外膜面积、厚度均较对照组显著增大(P<0.05),血管外弹力板围绕面积、内弹力板围绕面积和管腔面积较对照组显著减小(P<0.05),血管呈收缩性重塑。结论:血管成形术后,血管外膜被牵拉激活,导致外膜细胞分裂、增殖,以及血管收缩性重塑,参与再狭窄的发生。     

ST段抬高型急性下壁心肌梗死并心力衰竭患者的冠状动脉病变特征

目的:探讨ST段抬高型急性下壁心肌梗死(AIMI)并心力衰竭(HF)患者的冠状动脉病变特点及临床特征。方法:对108例行急诊冠状动脉介入治疗的ST段抬高型AIMI患者的临床资料进行分析,根据心功能分为2组,HF组32例,无HF组76例,比较2组临床特征及冠脉病变特点。结果:HF组年龄偏大(P<0.05),血清肌酐水平、恶性心律失常、心源性休克需要主动脉内球囊反搏比例(均P<0.01),三度房室传导阻滞发生率显著高于非HF组(P<0.05);罪犯血管为右冠状动脉,合并前降支血管病变,HF组显著高于非HF组(P<0.05和P<0.01)。结论:ST段抬高型AIMI罪犯血管为右冠状动脉,同时合并有前降支血管病变时,易发生心力衰竭。