Bent knees and tiptoeing: late manifestations of end-stage Parkinson's disease.

We describe a unique gait phenomenon of bent knees in 9 patients with idiopathic Parkinson disease (mean age, 73.1 +/- 11.1 years), 3 of whom also manifested tiptoeing. The bent-knee posture appeared only during ambulation; in the recumbent position, full or nearly full extension was possible in all patients. The abnormality emerged after long-standing disease (6-23 years from onset) and failed to respond to dopaminergic treatment. Most of the patients also had bent spine (camptocormia). The pathogenesis of these phenomena are unknown, but they might represent a rare type of dystonia.     

Ki67、P53及微血管密度在大肠肿瘤中的表达

目的 研究Ki67、P53及微血管密度(microvessel density,MVD)在大肠肿瘤中的表达,探讨其与大肠肿瘤癌变的关系及作为早期癌变生物学标志物的可能性。方法 采用免疫组化技术分别测定正常大肠黏膜、大肠息肉及大肠癌组织标本中Ki67、P53及MVD值,共80例,分析其变化规律及相关性。结果 在正常黏膜、大肠息肉、大肠癌组中的Ki67标记指数逐渐增高(分别为11.00±10.70、39.64±17.70、52.96±26.40),组间比较差异显著(P=0.0001)。正常黏膜组P53蛋白均为阴性,大肠癌组P53蛋白表达的阳性率明显高于息肉组(P=0.0001)。Ki67、P53蛋白表达与性别、年龄、病程、病变部位、大小、大肠癌的病理类型、Dukes分期均无相关性。正常黏膜、大肠息肉、大肠癌组的MVD值(14.80±5.10、19.70±7.84、36.56±20.40)逐渐上升,3组差别显著(P=0.0001)。大肠癌中Dukes C、D期的MVD值(40.56±3.49)明显高于A、B期(29.50±2.45)(P=0.016)。Ki67与P53在各种组织中的表达呈正相关(r=0.5149,P=0.015)。联合检测Ki67及P53鉴别大肠良恶性病变的敏感度(70.37％)低于单侧Ki67(96.30％),但特异度(94.34％)明显增高。结论 Ki67标记指数可反映细胞增殖状态,指数高的大肠腺瘤易发生癌变。MVD值高的大肠癌易发生转移,MVD可作为判断大肠癌预后的参考指     

不同湿热环境下颅脑火器伤死亡因素分析

目的　研究高温高湿环境下颅脑火器伤后生命体征变化特点及死亡因素。　方法　杂种猫 3 2只随机分为 4组 ,火器伤后分别放入A (2 5℃、相对湿度 50 % )、B (3 5℃、相对湿度85% )、C(3 8℃、相对湿度 90 % )、D(40℃、相对湿度 95% )四种环境中。每隔 10min记录一次血压、脉搏、呼吸频率及体温 ,到 6h时结束实验。　结果　常温枪伤组实验过程中生命体征稳定 ,随着温度及湿度的增加 ,各组动物相同时相点的体温、呼吸及心率均明显不同 (P <0 .0 5)。A组、B组动物在伤后 6h内均无死亡。C组 1.5h开始死亡 ,至 5h动物全部死亡 ,平均生存时间为 3 .4h。D组 0 .5h开始死亡 ,至 2 .5h动物全部死亡 ,平均生存时间为 2 .0h。　结论　高温高湿环境对颅脑火器伤时的生命体征变化和死亡率影响显著     

LONG‐TERM OUTCOME OF ENDOSCOPIC BALLOON DILATION IN OBSTRUCTIVE GASTROINTESTINAL CROHN's DISEASE

Background: Gastrointestinal strictures are the most often and serious complication in Crohn's disease. Because of the frequent postoperative recurrence in Crohn's disease, endoscopic therapy of gastrointestinal stricture is one of the best therapeutic options. Method: The present study sets out the results from a prospective study of endoscopic dilation therapy on 48 Crohn's disease patients with severe gastrointestinal stenoses. All patients who could not undergo endoscopic balloon dilation therapy (EBD) were operated on. Results: Long‐term success was attained in 32 of the 48 patients; cumulative avoidance of surgery after EBD was 86% at one year and 71% at three. Second, the most hazardous factor was recurrent inflammation causing restenosis. Patients who had strictures with oral luminal dilatation and patients with frequent recurrence had a tendency to be operated on. As a complication, perforation occurred in two cases (3.3%). Conclusions: EBD therapy for Crohn's stricture in the gastrointestinal tract is recommended before surgical intervention.     

Sleep complaints and their relation with drug treatment in patients suffering from Parkinson's disease.

The aim of this research was to quantify sleep problems in patients suffering from Parkinson's disease by means of the new Parkinson's Disease Sleep Scale (PDSS) and to correlate such problems with the possible influence of current drug treatment. A total of 70 patients (36 men and 34 women) with a diagnosis of Parkinson's disease were enrolled. Their mean age was 69.7 +/- 8.2 years, and duration of disease was 7.4 +/- 4.8 years. All patients completed the PDSS and the Unified Parkinson's Disease Rating Scale (UPDRS Parts I-IV). Drug consumption and doses were registered. The mean score on the PDSS scale was 109.23 +/- 19.75 and on the UPDRS III scale was 25.24 +/- 11.35. The lowest scores were obtained in Item 3 (sleep fragmentation): 5.53 (2.46); and in Item 8 (nocturia): 5.75 (2.91). There was a weak correlation between the PDSS and UPDRS III (cc = -0.355, P = 0.003), PDSS and UPDRS I (cc = -0.272, P = 0.02), and PDSS and UPDRS IV (cc = -0.416, P < 0.001). Motor conditions, mental state, and drug complications influence sleep quality. Although this effect was significant, it was not of a great magnitude. Dopaminergic drugs did not increase daytime sleepiness. As a whole, sleep quality in patients who took dopaminergic agonists did not differ from that of patients who took levodopa in monotherapy.     

G2019S LRRK2 mutation in familial and sporadic Parkinson's disease in Russia.

Among mutations associated with autosomal dominant and sporadic Parkinson's disease (PD) the G2019S substitution in the leucine-rich repeat kinase 2 (LRRK2) gene is the most frequently identified. To estimate its frequency in Russia, we analyzed 208 patients with PD from the Northwestern region of Russia. Of these, 51 patients were probands from families with PD compatible with autosomal dominant inheritance. The control group represented 161 subjects without neurological disorders settled in the same region. The frequency of the G2019S mutation was greater in familial PD (2 [3.9%] of 51) than in sporadic PD (1 [0.6%] of 157). In addition, this mutation was found in the proband's father, who also had PD, in 1 PD family, and in 1 carrier without signs of PD at age 40 in another PD family. All carriers were heterozygous for the G2019S mutation and reported the Ashkenazi Jewish origin. The mutation was not found in the control group.     

Chromatic pattern-reversal electroretinograms (ChPERGs) are spared in multiple system atrophy compared with Parkinson’s disease

Abstract Idiopathic Parkinson’s disease (IPD) patients have abnormal visual evoked potentials (VEPs) and pattern electroretinograms (PERGs), attributed to dopaminergic transmission deficiency in visual pathway, probably the retina. VEP abnormalities are not reported in multiple system atrophy (MSA). The aim of this study was to investigate and compare chromatic (Ch) red-green (R-G) and blue-yellow (B-Y), and luminance yellow-black (Y-Bk) PERGs in patients with MSA and IPD. We investigated 6 MSA patients (mean age: 62±7.4 years) not undergoing any pharmacological treatment, as well as 12 early IPD patients (mean age: 60.1±8.3 years) and 12 age-matched normal observers. ChPERGs were recorded monocularly in response to full-field equiluminant R-G, B-Y and Y-Bk horizontal gratings. In MSA only responses to R-G stimuli showed minimal insignificant changes (slight but not significant amplitude reduction without any significant latency delay); no significant abnormality was detected for B-Y and luminance Y-Bk stimuli. By contrast, in IPD all responses were reduced in amplitude and delayed in latency, above all for B-Y stimuli. Present data indicate that both chromatic and achromatic PERGs are virtually unaffected in MSA, whereas in early IPD they are clearly impaired, suggesting different pathogenic retinal mechanisms and a useful simple tool for distinguishing MSA from IPD.     

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.     

The Parkinson-Control study: a 1-year randomized, double-blind trial comparing piribedil (150 mg/day) with bromocriptine (25 mg/day) in early combination with levodopa in Parkinson's disease.

Dopamine agonists have been recommended as early treatment for Parkinson's disease (PD), alone or combined with levodopa. Piribedil is a non-ergot selective D(2)/D(3) agonist with alpha(2) antagonist properties shown to be effective in the treatment of PD. This 12-month international, randomized, double-blind trial aimed to assess the efficacy of piribedil 150 mg versus bromocriptine 25 mg, in early combination with levodopa in Stage I to III PD patients. Motor efficacy was assessed using the Unified Parkinson's Disease Rating Scale (UPDRS III, Items 18-31) as improvement from baseline. Response rate was defined as a 30% improvement. Among the 425 randomly assigned patients, 178 were also included in a substudy on cognitive follow-up evaluated by a dysexecutive syndrome oriented battery. A relevant improvement in UPDRS III over the 12-month study duration was observed both in the piribedil and bromocriptine groups (-7.9 +/- 9.7 points from baseline versus -8.0 +/- 9.5; not significant [n.s.]) with a response rate of 58.4% and 55.3% (n.s.), respectively. Piribedil and bromocriptine resulted in similar improvement on all UPDRS III subscores. Piribedil patients required less levodopa dose increase than those on bromocriptine. Cognitive performance remained generally unchanged in both groups, with a significant effect of piribedil limited to the Wisconsin Card Sorting Test. An overall good tolerability of piribedil was observed. Early combination of piribedil 150 mg with levodopa resulted in significant long-term improvement of all motor symptoms in PD patients insufficiently controlled by levodopa alone. Taking into account both efficacy and acceptability in the long-term, piribedil proved in this bromocriptine controlled study to be an effective and safe treatment for PD.     

Hydroxyethyl starch induced acquired von Willebrand's disease

Hydroxyethyl starch (HES) (Hespan, DPont) is a widely used synthetic volume expander which in standard doses of up to 1.51 in 24 h has no significant effect on coagulation (Munsch et al. 1988). However, the data sheet states that in large volumes HES may alter the coagulation mechanism. We now report a case of HES induced acquired von Willebrand's disease (vWD) in which severe bleeding occurred.