Ophthalmic surgery in prion diseases

Eleven (1.8%) of 597 patients underwent ophthalmic surgery within 1 month before the onset of prion disease or after the onset. All ophthalmologists reused surgical instruments that had been incompletely sterilized to eliminate infectious prion protein. Ophthalmologists should be aware of prion diseases as a possible cause of visual symptoms and use disposable instruments whenever possible.     

神经发育行为障碍儿童的睡眠障碍

神经发育障碍(NDDs)儿童合并睡眠障碍发生率高,其病程长短不一,治疗难度及复发率各方面均明显高于正常儿童。睡眠异常对神经发育行为障碍儿童在认知、情绪、社会发展和行为表现等多领域的损害也更为显著,甚至会严重影响家人的睡眠质量和生活质量。本文介绍NDDs儿童共患睡眠障碍的病因和危险因素及其相应的临床症状、评估手段以及干预办法,以期提高临床医师其对NDDs儿童睡眠障碍的早期识别及干预水平。通过改善睡眠质量来提高NDDs儿童的日间社会功能水平和康复治疗效果,并改善其家人的睡眠质量和生活质量。     

Sleep disorders in Parkinson's disease and other movement disorders

Neurodegenerative processes with movement disorders is predominant features show a high incidence of sleep alterations at some point in their evolution. The degeneration of structures responsible for maintaining the sleep-wakefulness cycles and the architecture of sleep could be at their root. Other factors like the drugs employed in the treatment of motor problems, the limitations to movement, etc., aggravate the problem. Although, at present, there is no medical therapy able to restore the defects derived from the degeneration of the key structures of sleep, an individual analysis of the coadyuvant factors in each patient could help to improve these problems. In this article we describe the main sleep disorders in Parkinson's disease and other degenerative diseases such as multi-system atrophies or progressive supranuclear paralysis.     

癫痫儿童睡眠障碍的研究进展

癫痫是一种儿童常见神经系统疾病,由脑部神经元高度同步化异常放电所致,以持久性的致痫倾向为特征.儿童癫痫和睡眠障碍相互影响,形成恶性循环.然而,目前临床医生对癫痫儿童的睡眠障碍重视不足,缺乏有效诊治.因此,本文就儿童癫痫和睡眠的相互影响、癫痫儿童常见的睡眠障碍,以及评估和治疗的方法进行论述,期望为癫痫儿童睡眠障碍的早期识...     

Sleep disorders in aging and dementia

This paper reviews the characteristics of sleep disorders found in people at a greater risk of dementia: the elderly adult, patients with mild cognitive impairment (MCI) and those with neurodegenerative diseases. The frequency of sleep architecture modifications and circadian rhythm sleep disturbances increases with age. Although around 40% of older adults complain of poor sleep, true sleep disorders are far less prevalent in healthy older adults and are frequently associated with comorbidities. The sleep disorders observed in Alzheimer’s disease (AD) patients are often similar to (but more intense than) those found in non-demented elderly people. Poor sleep results in an increased risk of significant morbidities and even mortality in demented patients and constitutes a major source of stress for caregivers. The prevalence of primary sleep disorders such as rapid eye movement (REM) sleep behavior disorders (RBDs), restless legs syndrome (RLS), periodic limb movements (PLMs) and sleep-disordered breathing increases with age. There are no published data on RLS and PLMs in demented persons but RBDs and sleep apnea syndrome have been studied more extensively. In fact, RBDs are suggestive of Lewy body dementia (LBD) and are predictive for neurodegeneration in Parkinson’s disease. Obstructive sleep apnea (OSA) shares common risk factors with AD and may even be an integral part of the pathological process in AD. In MCI patients, the hypotheses in which (i) sleep disorders may represent early predictive factors for progression to dementia and (ii) MCI is symptomatic of a non-diagnosed sleep disorder remain to be elucidated. Guidelines for drug and non-drug treatments of sleep disorders in the elderly and in demented patients are also considered in this review. In healthy but frail elderly people and in early-stage AD patients, sleep should be more thoroughly characterized (notably by using standardized interviews and polysomnographic recording).     

Prions, prion diseases and decontamination

Prions are extremely resistant to disinfection and sterilization methods used so far. The pathogenic prion protein core (called prion) consists of 142 amino-acids, is resistant to proteolytic enzymes, has a mass of 15 pikograms and is filtrable. Fixed by desiccation or chemicals may retain infectivity for years. It survives dry heat at 200 degrees C for 1-2 hours. Prions are fixed to stainless steel within minutes and remain infectious for long periods. Their pathogenetic properties depend on tertiary spatial structure (conformation) which is specific and transmissible in experiment. The prion decontamination appears by far the most important area of the prion science because very little, or nothing, has been done in the majority of world hospitals to prevent iatrogenic transmission. The number of potentially infectious patients is not known. Therefore, patients undergoing neurosurgery, laryngeal or ophthalmic operations, orthodental treatments and even anaesthetic or endoscopic applications should be classified into risk groups, even if clinically priondisease inapparent. The use (or misuse) of disposable instruments is certainly not the final answer for all cases and classic decontamination procedures, if possible because of the character of medical devices, appear still of greatest importance. We consider the high pathogen safety (HPS) autoclave from FEDEGARI as the best actual equipment for the effective decontamination of prions in the hospital practice. The investment costs are moderate and the handling is simple but must be careful. It appears practicable even in small specialized units.     

Understanding the natural variability of prion diseases

Prion diseases are a heterogeneous group of disorders with an invariably fatal disease course. Although various etiologies have been proposed it is apparent that at least a subset of these diseases are of infectious nature. An essential part of the infectious agent, termed the prion, is mainly composed of an abnormal isoform (PrP(Sc)) of a host-encoded normal cellular protein (PrP(C)). The molecular details of the pathophysiology of this group of diseases are unclear but the conversion of PrP(C) to PrP(Sc) plays a fundamental role. In all human prion diseases, PrP(Sc) is deposited in the central nervous system. These disorders include sporadic, genetic and acquired Creutzfeldt-Jakob disease. The molecular classification of human prion diseases is important in order to understand underlying disease mechanisms and for the development of novel therapy protocols. Current classification systems are based on the assessment of clinical presentation, genetic investigations, neuropathological findings and biochemical analysis of PrP(Sc).     

Sleep disorders in the elderly and in dementias

Sleep disorders are very frequent in the elderly. Bearing in mind the growth of this population group in western societies, it is of great importance to understand the sleep diseases that affect them and what their treatment should be. On the other hand, it is in this age group where we find the majority of patients with dementias. The treatment of sleep disorders in these patients is complex. An adequate control of insomnia and of the excessive nocturnal activity that usually occurs in the advanced phases of dementias has an important social repercussion. This article summarises the peculiar characteristics of sleep disorders in the elderly as well as the diagnostic and therapeutic handling of sleep disorders in patients with dementias.     

Clinical findings and diagnosis in genetic prion diseases in Germany

To describe the clinical syndrome and diagnostic tests in patients with genetic prion diseases (gPD) in Germany. Clinical features, MRI, EEG, and CSF markers were studied in 91 patients (28 D178N, 20 E200K, 17 inserts, 13 V210I, 8 P102L, 5 E196K). Dementia (35 %) and ataxia (29 %) were the most common initial symptoms and signs. A wide variety and high frequency of neurological/psychiatric symptoms and signs was found during disease course in all patients independently of the type of the mutation. Psychiatric manifestations were frequent (87 %). Neuropsychological abnormalities were observed in 67 %, and aphasia was the most common disturbance (45 %). In E200K, V210I and D178N patients, visual/oculomotor deficits were followed by ataxia early in the disease. Dementia followed by ataxia at onset was common in patients with insert and E196K mutation. P102L patients had isolated ataxia over a longer time period followed by pyramidal signs. Dementia was present only late in the disease course. All clinical routine tests such as MRI, EEG and CSF tests were less sensitive than in sporadic CJD. We provide the first detailed analysis of clinical signs and symptoms in a large group of patients with gPD. Frequency of clinical symptoms and signs was similar in different mutations in a later disease course, but the sequence of occurrence may be of great diagnostic importance. CSF markers were shown to be more sensitive than MRI and EEG.     

石家庄市学前儿童睡眠问题调查研究

目的 了解石家庄市学前儿童睡眠问题的流行情况及相关因素。方法 采用儿童睡眠习惯问卷(The Children's Sleep Habit Questionnaire, CSHQ)和自编问卷对石家庄市区6所幼儿园445名儿童进行测查。结果 石家庄市学龄儿童睡眠问题流行率总体为76.3%, 各类睡眠问题的流行率分布于12.1%(睡眠呼吸障碍)至67.6%(睡眠阻抗)之间。高年级、父母文化程度高、居住面积充足、单独睡眠、养育观念一致的儿童的CSHQ得分较低(P<0.01或<0.05)。与国外群体相比, 此次调查对象的CSHQ得分较高(P<0.01或<0.05)。结论 石家庄市学前儿童睡眠问题较严重、不同睡眠障碍类型流行率变化较大;睡眠问题与年级、父母文化程度、睡眠环境、家庭养育等因素有关。     

Sleep apnea-hypopnea syndrome and cardiovascular diseases

Sleep apnea-hypopnea syndrome is characterised by daytime sleepiness, cognitive-behavioural, respiratory, cardiac, metabolic or inflammatory disorders, secondary to repeated obstruction of the upper airway during sleep. Numerous studies have shown that SAHS is associated with the presence of arterial hypertension and the development of cardiovascular diseases. Similarly, there is an excess of mortality associated with SAHS. An increase in the activity of the sympathetic nervous system and endothelial dysfunction are considered as mediating mechanisms, together with phenomena of oxidative stress, platelet aggregation and thrombosis.     

亚健康概念框架的文献评析

目的 了解我国警察队伍躯体亚健康的现状及其影响因素,为制定有效的干预措施提供依据。 方法 2015年9月 — 2016年4月,采用方便抽样法,对13个地区1 805名公安民警进行问卷调查。 结果 排除自我报告患病的34.2 %样本后,警察群体的躯体亚健康得分为（41.54 ± 7.98）分;不同特征警察躯体亚健康得分比较,年龄25～29岁警察得分最高为（40.96 ± 7.94）分,45～49岁得分最低为（38.96 ± 8.64）分;婚未婚警察得分为（43.36 ± 6.70）分高于已婚警察的（39.54 ± 8.43）分,警种为特警的得分最高为（42.14 ± 6.51）分,治安警与综合类警察得分最低为（38.45 ± 8.64）和（38.69 ± 8.29）分,警员及以下者得分最高为（41.56 ± 7.91）分,警督及以上得分最低为（39.33 ± 8.65）分;警龄 < 5年者得分最高为（40.80 ± 7.91）分,≥ 20年者得分最低为（37.85 ± 9.39）分;不同年龄、婚姻状况、警种、警衔和警龄的警察躯体亚健康状况差异均有统计学意义（均P < 0.05）;吸烟的警察躯体亚健康得分较低（39.31 ± 7.81）分,运动达标的警察躯体亚健康得分最高为（42.93 ± 7.62）分,完全没有运动的得分最低为（39.74 ± 8.07）分,不同生活行为方式的警察躯体亚健康状况差异有统计学意义（均P < 0.05）。皮尔逊相关分析,抑郁（r = – 0.40）、焦虑情绪（r = – 0.45）与警察躯体亚健康明显负相关（均P < 0.01）;回归分析显示,婚姻状况、抑郁、焦虑、运动（β = – 0.09、– 0.12、– 0.31、– 0.17,均P < 0.01）是警察躯体亚健康的影响因素。 结论 警察躯体亚健康状况明显,受多种因素影响,应提高警察心理健康水平和培养健康生活方式。     

Sleep disorders and daytime sleepiness in state police shiftworkers

Police, who work shifts, participate in both risky and delicate tasks. The authors investigated sleep habits, prevalence of sleep disorders, sleepiness on the job, and hypnotic drug intake (Benzodiazepines, Zaleplon, Zolpidem, or Zoplicone) in a population of Italian state police officers. This study was conducted with self-administered questionnaires. The investigation focused on the difference between 540 non-shiftworkers (413 males, 127 females) and 575 shiftworkers (483 males, 92 females). All individuals were between 20 yr and 39 yr of age. In shiftworkers, there was a higher prevalence of difficulty in initiating sleep; in addition, these individuals had a sleep latency that exceeded 20 min, and they experienced early awakenings. No significant differences in daytime sleepiness and drug intake existed between the 2 groups. Self-evaluation of the number of hours that individuals slept each night and during a 24-hr period revealed that shiftworkers required more sleep. The results indicated that shiftworkers experienced a lower quality of sleep than non-shiftworkers, but the former did not report increased daytime sleepiness or increased hypnotic drug intake (i.e., Benzodiazepines, Zaleplon, Zolpidem, or Zoplicone). Shiftworkers seemed to compensate for the poor quality of their sleep by sleeping for a greater number of hours during 24-hr periods than the non-shiftworkers. Perhaps the aforementioned compensation resulted from a prolonged recovery from shiftwork effects.