视频脑电图在儿童心因性发作性疾病诊断上的应用

目的探讨视频脑电图（Video-EEG）对儿童心因性发作性疾病诊断价值。方法36例经Video-EEG监测后诊断为儿童心因性发作的患儿，对其临床资料作回顾性分析。结果在Video-EEG监测期间36例患儿均至少有一次临床发作，同步脑电图记录未见癫痫波发放，结合病史诊断为儿童心因性发作。结论Video-EEG在儿童心因性发作性疾病诊断上有重要意义。     

儿童发作性睡病16例误诊分析

目的探讨发作性睡病的临床特征、诊断、治疗及预后。方法回顾分析16例发作性睡病患者的临床表现及误诊情况,采用哌醋甲酯治疗。结果确诊病例予哌醋甲酯治疗症状明显缓解。结论发作性睡病常在青少年期起病并持续终生,可影响其智力及行为发育,加强对该病的正确认识,早期诊断及治疗非常重要。     

视频脑电图对儿童非癫痫性发作的诊断意义

目的探讨儿童非癫痫性发作(non-epileptic seizures,NES)的临床特征及视频脑电图(vid-eo-EEG)对其诊断意义。方法对129例经video-EEG监测后诊断为NES的患儿临床资料进行回顾性分析。结果 129例NES中,非癫痫性强直发作38例,良性肌阵挛31例,多发性抽动症20例,屏气发作9例,偏头痛7例,情感性交叉擦腿动作7例,睡眠障碍5例,轻度胃肠炎半婴幼儿良性癫痫4例,癔病3例,良性非癫痫性婴儿痉挛2例,其他3例,其中NES合并癫痫3例,NES合并亚临床发作2例。结论 video-EEG对NES的诊断有重要意义。     

发作性睡病多次睡眠潜伏期试验对照分析

我院于2001年1月至2005年3月共收治发作性睡病患者15例,采用白天多次睡眠潜伏期试验和整夜多导睡眠脑电图描记,探讨其睡眠周期的紊乱特征,并作为临床诊断本病的客观指标之一,现报告如下。1资料与方法1.1一般资料:本组15例作为患病组,男性10例,女性5例,年龄8~45岁,病程1~16年,平     

发作性睡病二例报告

1病例报告 例1男,33岁.主因间断"瞌睡"发作11年,加重1年,于2010年4月28日来诊.患者于11年前间断出现不明原因"瞌睡",每2周～1个月发作1次,每次发作2～60 s,于交谈及驾车或乘车过程中发作数次,因发作不频繁未予重视,未就诊,后间断发作数年,1年前发作较以往频繁,每周发作数次,发作时间数秒钟至数分钟,一般不超过10 min,常于驾车途中发作,严重影响工作及生活.遂辗转就诊多家医院,给予扩血管药物,改善脑代谢药物治疗无效,服用抗癫(痫)药物1年亦无明显缓解.遂收入我院治疗.既往体健,无吸烟,偶饮酒,量少,无肝炎、结核等传染病史,有磺胺过敏史,无食物过敏史,无毒物接触史,无家族遗传性疾病史.患者自发病以来饮食尚可,睡眠欠佳,体质量无明显减轻.体格检查:生命体征平稳,神志清楚,言语流畅,发育正常,营养良好;心肺腹查体未见异常;颅神经未见明显阳性体征;无四肢瘫,颈部无抵抗,病理反射未引出.行白天过度嗜睡检查,即多次小睡潜伏期试验(MSLT).夜间多导睡眠图(PSG)监测示平均睡眠潜伏期缩短(≤8 min),经过充足的睡眠(≥6h)后,次日试验可见≥2次睡眠始发快速眼动期(SOREMPs).查血清人类白细胞抗原分型(HLA-DR2)呈阳性.查血常规、生化、免疫、脑脊液常规生化、头颅MRI均未见异常,脑电图轻度异常.颅多普勒(TCD)示右侧椎动脉流速稍慢.临床诊断:发作性睡病.给予哌甲酯18 mg,每日1次口服治疗,1个月后症状明显改善,后坚持服药,效果良好.     

小儿发作性睡病2例报告

病例1，男，5岁，因“走路时常摔跤半年”以我科就诊。近半年来患儿无明显诱因出现走路时易摔跤且小睡片刻，叫醒后又继续行走。无持物跌落及抽搐等症状，无头痛无秽语及挤眉弄眼、耸肩、无呕吐。数d发作1次，少数情况下1d发作1～2次，发作前无先兆。     

小儿头痛的脑电图及临床分析

我科自1988年开展脑电图(EEG)检查以来,至1999年7月共检查罹患“头痛”的患儿脑电图622人次,大多数系偏头痛,且均经CT检查除外了颅内器质性病变。现将其脑电图的特点及临床表现分析如下。     

麻古戒断致发作性睡病

1例41岁男性,间断吸食苯丙胺类兴奋剂麻古(主要含有甲基苯丙胺和咖啡因)2年余,1年前开始自行减少剂量,就诊前4周完全停止吸食。戒断15d患者出现昼间嗜睡,频繁发作,10～20min发作1次,每次持续数分钟至数十分钟,并多次出现"梦魇"现象。多导睡眠图示平均睡眠潜伏期缩短至80s。诊断为发作性睡病,考虑为停用麻古所致。鼓励患者继续坚持戒断,增加体力活动和视听刺激,以避免白天睡眠。给予阿米替林25mg晚间睡前口服。2周后,患者睡眠障碍消失。     

儿童良性枕叶癫痫的临床分析

目的探讨儿童良性枕叶癫痫的临床表现及脑电图特点，以提高临床诊断水平。方法回顾性分析26例确诊为良性枕叶癫痫患儿的临床表现及脑电图特点。结果儿童良性枕叶癫痫的发作形式有：（1）视觉症状；（2）运动症状：偏转发作、全身性强直阵挛发作、偏身抽搐；（3）自动症。脑电图特点：一侧或双侧枕叶或枕叶及周围脑叶出现痫性放电。结论儿童良性枕叶癫痫是一组较具特征表现的癫痫综合征。掌握临床表现及脑电图特点特点，常可作出正确诊断。     

The ICSD-3 and DSM-5 guidelines for diagnosing narcolepsy: clinical relevance and practicality

Narcolepsy is a chronic neurological disease manifesting as difficulty with maintaining continuous wake and sleep. Clinical presentation varies but requires excessive daytime sleepiness (EDS) occurring alone or together with features of rapid-eye movement (REM) sleep dissociation (e.g., cataplexy, hypnagogic/hypnopompic hallucinations, sleep paralysis), and disrupted nighttime sleep. Narcolepsy with cataplexy is associated with reductions of cerebrospinal fluid (CSF) hypocretin due to destruction of hypocretin peptide-producing neurons in the hypothalamus in individuals with a specific genetic predisposition. Updated diagnostic criteria include the Diagnostic and Statistical Manual of Mental Disorders Fifth Edition (DSM-5) and International Classification of Sleep Disorders Third Edition (ICSD-3). DSM-5 criteria require EDS in association with any one of the following: (1) cataplexy; (2) CSF hypocretin deficiency; (3) REM sleep latency ≤15?minutes on nocturnal polysomnography (PSG); or (4) mean sleep latency ≤8?minutes on multiple sleep latency testing (MSLT) with ≥2 sleep-onset REM-sleep periods (SOREMPs). ICSD-3 relies more upon objective data in addition to EDS, somewhat complicating the diagnostic criteria: 1) cataplexy and either positive MSLT/PSG findings or CSF hypocretin deficiency; (2) MSLT criteria similar to DSM-5 except that a SOREMP on PSG may count as one of the SOREMPs required on MSLT; and (3) distinct division of narcolepsy into type 1, which requires the presence of cataplexy or documented CSF hypocretin deficiency, and type 2, where cataplexy is absent, and CSF hypocretin levels are either normal or undocumented. We discuss limitations of these criteria such as variability in clinical presentation of cataplexy, particularly when cataplexy may be ambiguous, as well as by age; multiple and/or invasive CSF diagnostic test requirements; and lack of normative diagnostic test data (e.g., MSLT) in certain populations. While ICSD-3 criteria reflect narcolepsy pathophysiology, DSM-5 criteria have greater clinical practicality, suggesting that valid and reliable biomarkers to help standardize narcolepsy diagnosis would be welcomed.     

猫抓病15例临床病理特征

目的 探讨猫抓病(CSD)临床病理特征、病因及诊断要点,以提高对CSD的认识,减少漏诊和误诊。方法回顾我院1986～1999年间所收集的15例猫抓病患者淋巴结活检资料,对其进行临床病理分析。结果 15例猫抓病患者均有与猫密切接触史;临床表现以自限性局部(颈部7例,腋下8例,肘部3例)淋巴结肿大为主,沿淋巴引流方向规律分布;HE镜下病理学特点为淋巴结内坏死性肉芽样微脓肿形成;特殊染色显示血管壁及微脓肿中革兰氏阴性、嗜银性、不耐酸的多形性杆菌存在。结论 CSD是一种病程自限的细菌感染性疾病,临床特征和淋巴结活检病理特点辅以Warthin-Starry银染色对其诊断及鉴别诊断具有重要意义。     

353例小儿肾小球疾病的病理及临床分布特点

目的 了解小儿肾小球疾病病理与临床分布特点。方法 收集353例肾活检患儿资料进行回顾性分析。结果 353例患儿中诊断为紫癜性肾炎117例(33.1%),孤立性血尿94例(26.6%),原发性肾病综合征87例(24.6%),急性肾炎综合征23例(6.5%),其次还有乙肝相关性肾炎、狼疮性肾炎、孤立性蛋白尿、慢性肾炎等。肾小球疾病中以原发性肾小球疾病最为常见(206例,占58.4%),病理改变主要为:系膜增生性肾小球肾炎(MsPGN)66例,占32.0%; IgA肾病(IgA N)54例,占26.2%;轻微病变37例,占18.0%;其他还包括微小病变(MCD)、膜增生性肾小球肾炎(MPGN)、膜性肾病(MN)、毛细血管增生性肾小球肾炎等。继发性肾小球疾病以紫癜性肾炎最常见。结论 肾小球疾病以原发性肾小球疾病为主,临床诊断以孤立性血尿最常见,继发性肾炎以紫癜性肾炎最常见。     

Effects of protriptyline on vigilance and information processing in narcolepsy

Vigilance, memory function, and response latency on the Sternberg short-term memory scanning task were examined in eight narcoleptic patients on and off medication. Off medication, half of the patients demonstrated reduced vigilance and all displayed diminished automatic memory encoding and longer response latencies on the Sternberg memory scanning procedure relative to the treated condition. Protriptyline normalized vigilance in half of the patients, while response latency and automatic information processing significantly improved in all. These findings are discussed with regard to the potential effect of the medication on central nervous system arousal.     

早期宫颈癌15例临床诊治分析

目的 通过对15例早期宫颈癌的诊断和治疗进行总结，强调早期宫颈癌规范化手术及综合治疗的重要性。方法 对15例患者进行了规范化手术及综合治疗，并对早期宫颈癌手术范围、手术要点和综合治疗模式等进行分析。结果随访3年，无一例复发。结论 按临床分期标准不应随意扩大手术范围，手术操作必须规范，加强综合治疗，术后恢复快，并发症减少。     

儿童桥小脑角部位胶质瘤的临床诊治

目的探讨儿童桥小脑角胶质瘤的诊断、鉴别诊断、治疗和并发症的处理等问题。方法对第148医院和青岛市市立医院神经外科1997～2002年手术治疗的儿童桥小脑角胶质瘤13例临床资料进行回顾性分析。结果星形细胞瘤10例,髓母细胞瘤3例;肿瘤全切5例,次全切除4例,部分切除4例,病死1例。结论对儿童桥小脑角胶质瘤早期诊断、早期显微手术,术后辅以放疗、化疗等综合措施,将能进一步提高疗效。     

An overview of hypocretin based therapy in narcolepsy

Introduction: Narcolepsy with cataplexy is most commonly caused by a loss of hypocretin/orexin peptide-producing neurons in the hypothalamus (i.e., Narcolepsy Type 1). Since hypocretin deficiency is assumed to be the main cause of narcoleptic symptoms, hypocretin replacement will be the most essential treatment for narcolepsy. Unfortunately, this option is still not available clinically. There are many potential approaches to replace hypocretin in the brain for narcolepsy such as intranasal administration of hypocretin peptides, developing small molecule hypocretin receptor agonists, hypocretin neuronal transplantation, transforming hypocretin stem cells into hypothalamic neurons, and hypocretin gene therapy. Together with these options, immunotherapy treatments to prevent hypocretin neuronal death should also be developed.

Areas covered: In this review, we overview the pathophysiology of narcolepsy and the current and emerging treatments of narcolepsy especially focusing on hypocretin receptor based treatments.

Expert opinion: Among hypocretin replacement strategies, developing non-peptide hypocretin receptor agonists is currently the most encouraging since systemic administration of a newly synthesized, selective hypocretin receptor 2 agonist (YNT-185) has been shown to ameliorate symptoms of narcolepsy in murine models. If this option is effective in humans, hypocretin cell transplants or gene therapy technology may become realistic in the future.     

头痛患儿180例脑电图分析

目的 探讨脑电图(EEG)检查对小儿头痛病因的诊断和鉴别诊断的价值.方法 对180例头痛患儿进行EEG描记,记录其脑电活动情况并分析.结果 180例患儿中,EEG表现正常56例,异常124例,异常率68.89％,其中轻度异常78例,占43.33％;中度异常14例,占7.78％;高度异常2例,占1.11％;临界状态5例,占2,78％;痫样活动25例,占13.89％.头痛间歇期正常97例,异常率为46.11％,显著低于发作期(x2 =15.16,P＜0.05).结论 对头痛患儿行EEC检查有重要意义.     

Pharmacological management of narcolepsy with and without cataplexy

Introduction: Narcolepsy is an orphan neurological disease and presents with sleep-wake, motoric, neuropsychiatric and metabolic symptoms. Narcolepsy with cataplexy is most commonly caused by an immune-mediated process including genetic and environmental factors, resulting in the selective loss of hypocretin-producing neurons. Narcolepsy has a major impact on workableness and quality of life.

Areas covered: This review provides an overview of the temporal available treatment options for narcolepsy (type 1 and 2) in adults, including authorization status by regulatory agencies. First- and second-line options are discussed as well as combination therapies. In addition, treatment options for frequent coexisting co-morbidities and different phenotypes of narcolepsy are presented. Finally, this review considers potential future management strategies. Non-pharmacological approaches are important in the management of narcolepsy but will not be covered in this review.

Expert opinion: Concise evaluation of symptoms and type of narcolepsy, coexisting co-morbidities and patients´ distinct needs is mandatory in order to identify a suitable, individual pharmacological treatment. First-line options include Modafinil/Armodafinil (for excessive daytime sleepiness, EDS), Sodium Oxybate (for EDS and/with cataplexy), Pitolisant (for EDS and cataplexy) and Venlafaxine (for cataplexy (off-label) and co-morbid depression). New symptomatic and causal treatment most probably will be completed by hypocretin-replacement and immune-modifying strategies.