Diagnosing CADASIL using MRI: evidence from families with known mutations of Notch 3 gene

Clinical data and MRI findings are presented on 18 subjects from two families with neuropathologically confirmed CADASIL. DNA analysis revealed mutations in exon 4 of Notch 3 gene in both families. All family members with mutations in Notch 3 gene had extensive abnormalities on MRI, principally lesions in the white matter of the frontal lobes and in the external capsules. Of several family members in whom a diagnosis of CADASIL was suspected on the basis of minor symptoms, one had MRI changes consistent with CADASIL; none of these cases carried a mutation in the Notch 3 gene. MRI and clinical features that may alert the radiologist to the diagnosis of CADASIL are reviewed. However, a wide differential diagnosis exists for the MRI appearances of CADASIL, including multiple sclerosis and small-vessel disease secondary to hypertension. The definitive diagnosis cannot be made on MRI alone and requires additional evidence, where available, from a positive family history and by screening DNA for mutations of Notch 3 gene. Received: 17 February 1999 Accepted: 23 July 1999     

Cavernous dilatation of mesencephalic Virchow-Robin spaces with obstructive hydrocephalus

We describe two patients with mild ventricular dilatation, shown to have cystic spaces in the midbrain, which we interpreted as greatly enlarged Virchow-Robin spaces. We discuss the pathophysiology and the possible relations to the mild hydrocephalus. Received: 1 September 1999/Accepted: 22 October 1999     

Contrast enhancement of the cerebrospinal fluid on MRI in two cases of spirochaetal meningitis

We report two patients with meningitis due to spirochaetal infection, both of whom showed diffusely enhancing meninges around the brain and spinal cord. In addition, there was enhancement of the cerebrospinal fluid after intravenous administration of Gd-DTPA. Received: 1 March 1998/Accepted: 21 August 1999     

MRI of orbital schwannomas

The literature on MRI of orbital schwannomas is limited. The appearances in three patients with an orbital schwannoma were reviewed. A superior orbitotomy through a subfrontal craniotomy revealed a schwannoma in all cases. MRI characteristics of very low signal on T 1-weighted images and homogeneous postcontrast enhancement may be helpful for differentiating schwannomas from other intraconal masses. Received: 21 June 1999/Accepted: 20 September 1999     

局部药代动力学的药物浓度时空方程初探

目的 建立局部植入给药的药物浓度时空方程。方法 将局部药物浓度时空方程构建为空间分布函数和时间分布函数(药物浓度经时方程)的积函数。并以加权平均及多元分析的数学手段予以证明及推导。结果 四维时空的局部植入给药的药物浓度时空方程的通式为：C(t，X，Y，Z)＝F(X，Y，Z)．C(t)，F(X，Y，Z)＝B0 B1X B2Y B3Z B4r3 B5r2 B6r,C(t)＝A1e^-Ket A2e^-Ket A3c^-Ket.。两维变量的简化的药物浓度时空方程为：C(t,ρ)＝F(ρ)．C(t)F(ρ)＝D0 D1ρ D2ρ2 D3ρ3，C(t)同上。结论 推导基本合理，并在初步的动物试验探索中得以验证。理论有较好科研价值，有助于揭示局部给药的药代动力学规律。     

SPECT脑血流显像在难治性癫癎灶定位诊断中的价值

目的 :探讨单光子发射计算机断层(SPECT)脑血流显像在癫灶定位诊断 ,指导癫外科治疗的价值。方法 :对88例难治性癫患者进行了脑电图(EEG) ,磁共振成像(MRI)和SPECT脑血流显像检查 ,其中42例患者进行了外科(开颅手术或γ刀放射)治疗。结果 :SPECT脑血流显像的阳性率最高 ,为90.9 % ,EEG和MRI的阳性率分别为76.1 %和40.9 %。外科治疗患者SPECT与EEG定位结果的一致及相对一致率为83.3 % ,SPECT与MRI定位结果的一致及相对一致率为81.3 %。3者定位结果的一致及相对一致率为75.0 %。外科治疗的有效率为83.3 %。结论 :SPECT脑血流显像是诊断癫灶灵敏而有效的方法 ,对正确选择癫手术或γ刀治疗方案并取得较好的治疗效果具有重要意义     

迷路纤维化的诊断及人工耳蜗植入3例分析

目的：探讨迷路纤维化的影像学诊断及对人工耳蜗植入术的效果。方法：结合文献复习，报告3例迷路纤维化患者行人工耳蜗植入术的临床资料。结果：术前CT扫描3例均未发现异常。1例术前MRI提示耳蜗基底间纤维状，1例术后提示对侧耳蜗纤维化。2例病变较轻者成功地进行了人工耳蜗植入术，术后恢复良好；1例因病变严重电极未能植入。结论：MRI在迷路纤维化的诊断中有重要价值。慢性化脓性中耳炎为迷路纤维化的病因之一。慢性化脓性中耳炎一期手术清理中耳病变后，具有进行人工耳蜗植入的可行性。     

咪喹莫特脂质体与乳膏体外经皮扩散行为的比较

采用中和法制备咪喹莫特脂质体，研究了其体外经皮扩散行为并与咪喹莫特乳膏进行比较。制得的脂质体平均粒径为855nm，跨距为1．65，能经皮缓慢扩散，皮层的局部贮药量比之乳膏有增加，可减少药物全身吸收。     

1996～2001年内科、外科、ICU和门诊患者分离的金黄色葡萄球菌耐药性分析

目的 通过对1996～2001年60家三级甲等医院不同科别住院患者和门诊患者分离的金黄色葡萄球菌的耐药性分析，探讨不同科别来源菌株耐药率间的差别及变迁。方法 药敏试验采用纸片扩散法，用WHONET5软件分析结果。结果 6年间内科住院患者分离的金黄色葡萄球菌对苯唑陛西林的耐药率维持在40％；磺胺甲恶唑／甲氧苄啶(SMZ／TMP)和氯霉素的耐药率从1998年的51．4％和39．6％降低到2001年的36．2％和23．6％。外科住院患者分离菌株对苯唑西林的耐药率从1996年的26．3％增加到2001年的45．9％；庆大霉素和环丙沙星耐药率从1996年的24．6％和17．5％增到2001年的43．5％和43．1％。ICU患者分离的大多数菌株对苯唑西林和环丙沙星耐药。门诊患者分离菌株对苯唑西林的耐药率从1996年的17．2％增加到2001年的24．9％；环丙沙星的耐药率超过30％；SMZ/TMP的耐药率从1998年的61％降低到2001年的38．3％。结论 调查不同科别住院患者和门诊患者来源的金黄色葡萄球菌对常用抗生素耐药性的变迁和现状，对于临床抗感染治疗的经验用药具有一定的指导意义。