母亲苯丙酮尿症后代的脑MRI表现

目的 提高对母亲苯丙酮尿症(MPKU)后代脑MRI表现的认识。资料与方法 报告2例生化及临床检查证实为MPKU双胞胎的．MRI改变，并复习文献。结果 文中2例表现与文献报道不一致．与苯丙酮尿症患儿的表现相似。常规T2WI及FLAIR均显示室周白质、侧脑室三角区、半卵圆中心条片状高信号。弥散加权成像(DWI)除更清楚地显示上述改变外，尚显示双侧内囊后肢及胼胝体压部信号增高。结论 常规T2WI及FIJAIR上可显示MPKU后代脑白质异常，DWI可提供更多诊断依据。     

络合碘在预防阑尾炎术后切口感染中的作用

目的络合碘预防阑尾切除术后切口感染。方法0．05％络合碘冲洗切口65例。结果络合碘冲洗的化脓、坏疽或穿孔性阑尾炎切口无感染，与传统组感染率9．1％（6／66）比较p〈0.05（x^2=4．266）。结论0．05％络合碘冲洗切口是预防阑尾切除术后切口感染的有效方法。     

International multicenter pilot study of the first comprehensive self-completed nonmotor symptoms questionnaire for Parkinson's disease: the NMSQuest study.

Nonmotor symptoms (NMS) of Parkinson's disease (PD) are not well recognized in clinical practice, either in primary or in secondary care, and are frequently missed during routine consultations. There is no single instrument (questionnaire or scale) that enables a comprehensive assessment of the range of NMS in PD both for the identification of problems and for the measurement of outcome. Against this background, a multidisciplinary group of experts, including patient group representatives, has developed an NMS screening questionnaire comprising 30 items. This instrument does not provide an overall score of disability and is not a graded or rating instrument. Instead, it is a screening tool designed to draw attention to the presence of NMS and initiate further investigation. In this article, we present the results from an international pilot study assessing feasibility, validity, and acceptability of a nonmotor questionnaire (NMSQuest). Data from 123 PD patients and 96 controls were analyzed. NMS were highly significantly more prevalent in PD compared to controls (PD NMS, median = 9.0, mean = 9.5 vs. control NMS, median = 5.5, mean = 4.0; Mann-Whitney, Kruskal-Wallis, and t test, P < 0.0001), with PD patients reporting at least 10 different NMS on average per patient. In PD, NMS were highly significantly more prevalent across all disease stages and the number of symptoms correlated significantly with advancing disease and duration of disease. Furthermore, frequently, problems such as diplopia, dribbling, apathy, blues, taste and smell problems were never previously disclosed to the health professionals.     

嗜肺军团菌肺炎13例临床分析

本文报告１３例嗜肺军团菌肺炎。其中Ｌｐ１型６例；Ｌｐ６型６例；Ｌｐ１２型１例。Ｌｐ１２型国内尚未见文献报告。对临床表现，实验室检查，Ｘ线胸部征象，治疗和预后进行分析。并将部分资料与美国洛杉矶Ｗａｄｓｗｏｒｔｈ医疗中心统计资料分析对比，以探讨国人嗜肺军团菌肺炎临床特征。     

人群中病毒性肝炎的型别研究

作者于1987年2月16日～1988年2月15日在四川五个点211 639人群中监测急性病毒性肝炎。其发病率为167.74/10万,其中甲型肝炎占24.51％;乙型肝炎占38.31％;非甲非乙型肝炎占24.51％,EB病毒和巨细胞病毒所致肝炎各占3.38％;混合感染占5.92％。病毒性肝炎中有14.93％重叠感染。发病高峰在春季。发病率,男:女=1.75:1。甲型肝炎20岁以下年龄组、乙型肝炎20～39岁年龄组的发病率明显高于其他年龄组,非甲非乙型较分散,5～19岁相对多一些,其他型别年龄组间无明显差异。355例急性病毒性肝炎患者中43.9％有接触史,36.6％有注射史。     

龈沟液隐血试验与牙龈出血指数、龈下菌群的关系

目的 探索一种无创性的牙龈出血检测方法－－龈沟液隐血试验（GCFobt），并分析牙龈出血与微生物之间的内在联系。方法 用隐血试纸进行102例唾液标本的唾液隐血（Sobt）检查，1600个位点的GCFobt、牙龈出血指数（SBI）、探诊深度（PD）检测，分别对79、32个龈下细菌标本作涂片检查和厌氧培养，分析临床指数与龈下细菌的关系。结果 GCFobt具较高灵敏度（68.05）和特异度（80.5%），较Sobt更能说明局部牙周炎症状况，与SBI有非常显著相关关系（P＜0.001）；GCFobt阳性与阴性部位螺旋体、杆菌、球菌百分含量有非常显著差异（P＜0.001）；在0与3之间黑色菌检出率有非常显著差异（P＜0.01），在0与2、3之间梭杆菌检出率有显著差异（P＜0.05）。结论 GCFobt是一种快速、简便、无创伤的检测牙龈出血新方法，可以作为牙周病临床检查的一项客观指标。     

部分性脾栓塞术的临床应用：——附37例报告

应用部分脾栓塞术(PSE)治疗难治性原发性血小板减少性紫癜27例,血栓性血小板减少性紫癜1例;难治性再生障碍性贫血2例;Evan's 综合症2例;系统性红斑狼疮合并血小板减少性紫癜2例;继发性脾亢3例.获完全缓解者24例(64.9%);部分缓解者8例(21.6%),总有效率为86.S%.24例获完全缓解者18例追踪1年,6例复发,复发率为33.3%.与25例切脾对照组及文献报道比较,差异无显著性(P>0.05).本文对 PSE 术后外周血小板的变化进行讨论,并与脾切除作了对比。本文还对血小板破坏场、栓塞范围和方法等影响疗效的因素进行了探讨.作者认为 PSE 术可作为脾切除的替代术.     

Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS): Process, format, and clinimetric testing plan.

This article presents the revision process, major innovations, and clinimetric testing program for the Movement Disorder Society (MDS)-sponsored revision of the Unified Parkinson's Disease Rating Scale (UPDRS), known as the MDS-UPDRS. The UPDRS is the most widely used scale for the clinical study of Parkinson's disease (PD). The MDS previously organized a critique of the UPDRS, which cited many strengths, but recommended revision of the scale to accommodate new advances and to resolve problematic areas. An MDS-UPDRS committee prepared the revision using the recommendations of the published critique of the scale. Subcommittees developed new material that was reviewed by the entire committee. A 1-day face-to-face committee meeting was organized to resolve areas of debate and to arrive at a working draft ready for clinimetric testing. The MDS-UPDRS retains the UPDRS structure of four parts with a total summed score, but the parts have been modified to provide a section that integrates nonmotor elements of PD: I, Nonmotor Experiences of Daily Living; II, Motor Experiences of Daily Living; III, Motor Examination; and IV, Motor Complications. All items have five response options with uniform anchors of 0 = normal, 1 = slight, 2 = mild, 3 = moderate, and 4 = severe. Several questions in Part I and all of Part II are written as a patient/caregiver questionnaire, so that the total rater time should remain approximately 30 minutes. Detailed instructions for testing and data acquisition accompany the MDS-UPDRS in order to increase uniform usage. Multiple language editions are planned. A three-part clinimetric program will provide testing of reliability, validity, and responsiveness to interventions. Although the MDS-UPDRS will not be published until it has successfully passed clinimetric testing, explanation of the process, key changes, and clinimetric programs allow clinicians and researchers to understand and participate in the revision process.     

Integrated home monitoring predicts lead failure in a pacemaker dependent 4-year-old girl.

A 4-year-old girl with post-surgical complete atrioventricular block received an epicardial dual chamber pacemaker system. During further growth intermittent exit block occurred, first misinterpreted as neurological seizures. The epicardial lead was replaced using a transvenous approach, and a pacemaker with an integrated home monitoring facility was implanted. After her discharge, a rise in the pacing threshold automatically initiated an event message. On the basis of this information, the patient was called in and imminent dislodgement of the ventricular lead was diagnosed by x-ray. The lead was repositioned and was found stable over 1-year follow-up.     

Case report: Concordant traumatic brainstem contusion delayed diagnosis in a young man with Wilson's disease

Wilson's disease is a hereditary autosomal recessive disorder of copper metabolism. The corresponding gene locus has been localized on the long arm of chromosome 13. Three different clinical variants of the disease can be distinguished: hepato-cerebral, abdominal/hepatic, and central nervous type. The heterogeneity of symptoms can cause problems in differential diagnosis, especially when another concordant disorder can also explain the pathogenesis of symptoms. The case report of a young man who suffered from brainstem contusion demonstrates the possibilities of misinterpretation because presenting symptoms could be attributed either to traumatic brain injury followed by adjustment disorder or Wilson's disease. Clinical signs included leftsided hemiparesis, bilateral gaze direction nystagmus, marked dysarthria with consecutive pervasive mutism, choreo-athetoid movements, spasmodic torticollis and diplopia dependent on gaze direction. Slit lamp examination showed Kayser-Fleischer's corneal ring. EEG- and computer assisted tomography investigations revealed non-specific findings. The patient was treated with D-Penicillamine. Alternative treatment with oral zinc preparations is discussed.

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Zusammenfassung Beim Morbus Wilson handelt es sich um eine autosomal rezessiv vererbte Störung des Kupferstoffwechsels. Der Genort konnte auf dem langen Arm des Chromosoms 13 lokalisiert werden. Klinisch können aufgrund ihrer Symptomatik drei Verlaufsformen (hepato-zerebraler, abdominalhepatischer und zerebraler Typ) unterschieden werden. Die Vielfalt der Symptome kann differentialdiagnostische Schwierigkeiten bereiten. Das Beispiel eines jungen Mannes mit einer traumatischen Hirnstammkontusion zeigt, wie die Diagnose der hepato-lentikulären Erkrankung dadurch erschwert wurde, daß die Pathogenese der Symptome durch die Hirnstammkontusion und darauf folgende Anpassungsstörungen erklärt worden war. Die Symptomatik bestand aus linksseitiger Hemiparese, lateralem Blickrichtungsnystagmus, Dysarthrie mit nachfolgendem universalem Mutismus, choreo-athetodischen Bewegungsstörungen, Torticollis spasmoidicus und blickrichtungsabhängigem Auftreten von Doppelbildern. Bei der Spaltlampenuntersuchung stellte sich der Kayser-Fleischer Ring dar. EEG- und computertomographische Untersuchungen erbrachten nur unspezifische Befunde. Die Behandlung erfolgte mit D-Penicillamin. Die alternative Behandlung mit oraler Gabe von Zinksalzen wird diskutiert.

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Résumé La maladie de Wilson est une affection héréditaire autosomale recessive concernant le métabolisme cuivré. Le locus du gène a été situé sur le bras long du chromosome 13. Du point de vue clinique on distingue trois formes symptomatologiques: le type hepato-cérébral, hepato-abdominal et cérébral. La diversité des signes cliniques peut poser des problèmes de diagnostic différentiel, car d'autres affections peuvent se présenter avec cette même Symptomatologie. Nous rapportons ici l'exemple d'un homme jeune, porteur d'une maladie de Wilson et victime d'une contusion traumatique du tronc cérébral, dont les signes cliniques ainsi que les troubles du comportement pouvaient été autant rapportés à la contusion du tronc cérébral qu'à l'affection métabolique.La Symptomatologie comprenait une hemiparesie gauche, un nystagmus lateralisé, une dysarthrie avec mutisme secondaire universel, des mouvements choréo-athétosiques, un torticolis spasmodique et une diplopie dépendante de la direction du regard. L'examen à la lampe à fente permettait à mettre en evidence un anneau de Kayser Fleischer. L'EEG et le scanner cérébral ne montraient pas d'anomalies specifique.Le traitement a consisté en l'administration de D-Penicillamine. Traitment alternative avec les sels de zinc est discuté.