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1、多发性硬化及急性炎症性脱髓鞘多发性神经病康复,康复科锁冬梅,Definition,Multiple sclerosis (MS) is a chronic autoimmune disease that begins most commonly in young adults and is characterized pathologically by multiple areas of central nervous system (CNS) white matter inflammation, demyelination, and glial scarring (sclerosis). T
2、he lesions are therefore multiple both in space and time Incidence 530/100,000; F:M=1.53.0:1 Eastern world:1.4/100,000,多发性硬化 (multiple sclerosis, MS) 是以中枢神经系统白质脱髓鞘病变为特点的自身免疫病。 临床特征: 病灶时间上、空间上的多发性,髓鞘(myelin) 是包绕神经轴突周围的一种胶质细胞膜性脂质结构,中枢 少突胶质细胞 MS 周围 Schwann细胞 GBS,Basic Knowledge,Neuron Myelin and Demyel
3、inate created by glia Fatty substance Support , nutrition, insulate , speeds transmission MS plaque is defined as a sharply area of demyelination with glial proliferation,What Causes Multiple Sclerosis,Immunology: MS involves an auto-immune processan abnormal response by the immune system directed a
4、gainst the central nervous system (CNS). MS patients immune system cannot differentiate between virus proteins and its own myelin and therefore launches an attack against itself Viral: it is possible that a virus is the triggering factor in MS. More than a dozen viruses have been investigated , unfo
5、rtunatly no one is sure,What Causes Multiple Sclerosis,下一个会不会是我,别杀我，是自己人,杀的就是你,嘻嘻,Geographic incidence of MS, 1965, McAlpine,Clinical features-The initial symptoms,The symptoms and signs are diverse and seem to include all the symptoms that can result from injury to any part of the neuraxis from the
6、 spinal cord to the cerebral cortex. The chief characteristics are multiplicity and tendency to vary in nature and severity with time. difficulty in walking abnormal sensations such as numbness or a feeling of “pins and needles“ pain and loss of vision due to optic neuritis (inflammation of the opti
7、c nerve),Clinical features-Other symptoms,dizziness and vertigo dysphagia (difficulty in swallowing) headache hearing problems spasticity (stiffness) Tremor itching,numbness pain seizures loss of sexuality or sexual problems Bowel and bladder incontinence Lhermitte sign,Clinical Courses (type of MS)
8、 -RRMS(Relapsing/Remitting MS),relapses (exacerbations) : new symptoms can appear and old ones resurface or worsen. The relapses are followed by periods of remission, fully or partially recovers. Relapses can last for days, weeks or months and recovery can be slow and gradual or almost instantaneous
9、. The vast majority of people are RRMS, especially in early age, F/M=2:1.,SPMS (Secondary Progressive SPMS),50% RRMS will pass into a SPMS after 10 years. worsening of the disease with no periods of remission in between.Early phase may have relapse. But,in end-phase, it merge into a general progress
10、ion.,PRMS ( Progressive Relapsing Multiple Sclerosis PRMS ),This form of MS follows a progressive course from onset. between relapses there is a gradual worsening of symptoms.,PPMS ( Primary Progressive PPMS),gradual progression of the disease from its onset with no remissions at all. PPMS differs f
11、rom RRMS and SPMS in that onset is typically in the late thirties or early forties, men are as likely women to develop it and initial disease activity is in the spinal cord and not in the brain.,Diagnosis Criteria INTERNATIONAL PANEL REVISES DIAGNOSTIC CRITERIA FOR MS,Diagnosis Criteria,Diagnosis Cr
12、iteria,Treatment-Symptoms,Fatigue ：amantadine (金刚烷胺)， Spasticity ： exercise for mild cases baclofen or diazepam for severe Bladder problems ：Banthine（溴苯新） Maprotiline (麦普替林) Tremor ：carbamazepine (卡马西平) Visual problems ：methylprednisolone Emotional disorders ：amitriptyline (阿米替林)，desipramine (去甲丙咪嗪)
13、 imipramine (丙咪嗪),EDSS评分系统,EDSS也叫扩大的病残状态分级 ( Expanded disability status scale ,EDSS) 是目前在国际上通用于MS 的残损及残障的评估系统 该系统可以对神经各系统(锥体、小脑、脑干、感觉、直肠及膀胱, 大脑) 进行定量分析，有助于病情变化和治疗效果的评估,发展历史,1955年 Kurtzke制定了Disability Status Scale (DSS)量表，将MS损害分为0级（正常）10级（死于MS) DSS分级过粗，不能区别疾病严重性的微小变化， 1983年在每相邻两级间增加了半个级别，修订为0、1、1.510
14、共20个级别，故称EDSS量表,EDSS评分系统,功能 系统 积分 （FS）,EDSS 积分 评定,锥体系统 小脑功能 脑干功能 感觉功能 直肠或膀胱功能 视觉功能 脑（精神）功能 其他功能,0分 1分 1.5分 10分,量表一：功能系统积分量表,量表一：功能系统积分量表,各系统的功能评定相对于其他系统都是独立的 每一个系统都可以过一段时间再评定，以前后对比 各系统综合起来可反映整个神经系统的情况 该量表不是多余的，便于分各个系统观察功能受损情况,量表二：EDSS积分评定量表,03.5分是在各功能系统评定量表的基础上得出的 4-5.5分是患者在无须帮助的情况下行走的距离 67.5分是患者行走时
15、受帮助的情况 89.5分关助患者使用上肢、自我照顾、交流、吃饭的能力,量表二：EDSS积分评定量表,评分的前几步中，症状的少量增加就可以导致EDSS评分明显增加。这意味着病变累及了更多的系统或某一系统的功能障碍比较严重 第四步之后，行走能力是决定EDSS分值的主要因素。评分的这一部分中，其他功能的异常，对EDSS评分的影响不大，尽管这些功能（如上肢的运动、认知能力）对患者本人有一定影响NMO课题评定表EDSS量表.doc,EDSS评分系统的优缺点,优点是临床应用广泛，评价方便 缺点是在某些方面具有主观性，如二便功能 过度关注行走方面的功能，对于感觉认知等方面不能很好的反映出来 对于一些程度副词
16、如“轻度、中度、重度”未严格限定，不同评定者评分结果不一,康复治疗,一.临床处理原则 二康复治疗原则与方法,康复治疗方法,1 关节活动范围方法医专神经康复神经康复学ppt脑卒中康复12 脑卒中康复.ppt 2 肌力训练 3痉挛状态 4疲乏 5震颤和共济失调 6感觉障碍 7疼痛 8膀胱直肠功能障碍,康复治疗方法,9构音障碍和吞咽障碍 10视觉功能障碍 11发作性症状的处理 12认知障碍 13精神与情绪障碍 14社会心理和职业障碍,急性炎症性脱髓鞘性多发性神经病康复,一、定义,A1DP又称急性感染性多发性神经根神经炎、急性炎性多发性神经病、吉兰-巴雷综合症（GBS），是一种原因不清的引起急性或亚急性瘫痪的疾病，以周围神经脱髓鞘及血管周围炎症反应为病理特点的自身免疫病。因近年来发现了不少急性轴索变性病例，所以GBS这一术语似更合适。,二、简史： 1859年 法国Landry O 报道为“急性上升性麻痹” 1892年 美国OslerW 具体描述了该病 1916年 Guillain , Barre,Strohl报道了2例伴有腱反射丧失的急性瘫痪士兵，强调了脑脊液表现及病理，被后人称之Landry-Guillain-BarreStrohl syndrome,
