錐體外系疾病2-運(yùn)動障礙性ppt課件

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1、錐體外系疾?。ㄟ\(yùn)動障礙性疾?。〦xtrapyramidal diseases ( movement disorders)神經(jīng)病學(xué)教研室 Department of Neurology Zhijian Zhang,錐體外系疾病, 主要表現(xiàn)為隨意運(yùn)動調(diào)節(jié)功能障礙，肌力、Extrapyramidal diseases mainly impair the regulation of voluntary motor activity without,感覺及小腦功能不直接受影響。運(yùn)動障礙疾病源于基底節(jié)功能紊亂。directly affecting strength, sensation, or cereb

2、ellar function. Movement disorders result from dysfunction of deep subcortical gray matter structures termed the basal ganglia.,解剖：Anatomy:基底節(jié)：尾狀核、豆?fàn)詈耍ず恕⑸n白球）、屏狀核和杏仁復(fù)合體。Basal ganglia: caudate nucleus, lentiform nucleus ( putamen, globus pallidus), claustrum and amygdaloid complex.,新紋狀體：殼核、尾狀核。Neostr

3、iatum: putamen, caudate.舊紋狀體：蒼白球Paleostiratum: globus pallidus古紋狀體：杏仁復(fù)合體Archistriatum: amygdaloid complex,相關(guān)結(jié)構(gòu)：紅核、黑質(zhì)、丘腦底核、中腦間質(zhì)核、大腦皮質(zhì)的4、6區(qū)。Dependency structure: red nucleus ( corpus rubrum), substantia nigra, mid brain interstitial nucleus, 4 and 6 regions of cerebral cortex,神經(jīng)環(huán)路：Neuronal loops: 1.皮質(zhì)

4、-皮質(zhì)環(huán)路：大腦皮質(zhì)-尾狀核、殼核-內(nèi)側(cè)蒼白球-丘腦-大腦皮質(zhì)1. Corticortical loop: cerebral cortex-caudate, putamen-the internal segment of the globus pallidus-thalamus-cerebral cortex,2 .黑質(zhì)-紋狀體環(huán)路：黑質(zhì)與尾狀核、殼核之間的往返聯(lián)系纖維2. Nigrostriatal loop: connecting the substantia nigra, caudate and putamen,3. 紋狀體-蒼白球環(huán)路：尾狀核、殼核-外側(cè)蒼白球-丘腦底核-內(nèi)側(cè)蒼白球3.

5、 Striaropallidal loop: projects from the caudate and putamen to the external segment of the globus pallidus, then to the subthalamic nucleus, and finally to the internal segment of globus pallidus.,,,神經(jīng)遞質(zhì)：乙酰膽堿（Ach）、多巴胺 (DA)、去甲腎上腺素 (NE)、5-羥色胺 (5-HT)、-氨基丁酸 (GABA) 、谷氨酸等。Neurotransmitter(NT): acetylchol

6、ine, dopamine, noradrenaline, 5- hydroxytryptamine(serotonin), gamma-aminobutyric acid, aminoglutaminic acid, etc.,臨床分類：Clinical classification:1運(yùn)動過多：震顫、肌張力障礙、舞蹈癥、手足徐動癥、抽搐1. hypercinesia: tremor, dysmyotonia, chorea, posthemiplegic chorea, tic2運(yùn)動減少：帕金森病和帕金森綜合征。2. hypokinesia: Parkinsons disease and

7、 Parkinsons syndrome,3. 其他疾?。汗矟?jì)失調(diào)、肝豆?fàn)詈俗冃浴?. Other diseases: ataxia, Kinnier-Wilson syndrome治療原則：Therapeutic principle: 病因治療、藥物治療、神經(jīng)外科治療、個體化原則。Etilogical treatment，drug treatment，neurosurgical therapy, individualized treatment,帕金森病 ( agitans paralysis; paralysis agitans; Parkinsons disease; PD),帕金森病又

8、名震顫麻痹，是一種常見的中老人神經(jīng)系統(tǒng)變性疾病，50歲以上Parkinsons disease, also named Paralysis agitans, is a kind of frequent nervous system,多見。發(fā)病率20/10萬/年?；疾÷?60/10萬/年。70歲人群達(dá)120/10萬/年。degenerative disease in middle and old age, it mostly occurs in the age of above 50. The attack rate is 20/100 thousand population per year,

9、 the morbidity rate is 160/100 thousand population per year, and is 120/100 thousand per year in the population of about 70 years old.,病因與病機(jī)：etiological factor and pathogenesis: 病因不明。發(fā)病可能與以下因素有關(guān)： etiology unknown. The factors bellow possibly relates the onset:,1. 年齡老化：主要發(fā)病為中老年人，但只是促發(fā)因素。Ageing: it ma

10、inly attacks aged people, but the ageing is only the precipitating factor.,2.環(huán)境因素：MPTP使ATP生成減少，自由基生成，氧化應(yīng)激反應(yīng)，導(dǎo)致多巴胺能神經(jīng)元變性死亡。 Environmental factor: The MPTP reduces the generation of ATP, preciptitates the generation of free radical and increases the oxidative stress, which results in the degeneration

11、and death of dopamine neurons.,,,,,3.遺傳因素：10%-15%病人有家族史，多呈常染色體顯性遺傳。Hereditary factor: 10-15 percent patients have the family history, and most of them belong to autosomal dominant inheritance.,4. 其他：氧自由基、羥自由基、興奮性氨基酸。目前認(rèn)為是多因素作用的結(jié)果。Other factors: free radical, hydroxy radical, excitatory amino acids.R

12、ecently the occurence of this disease is considered as the effective result of multiple factors,病理與生化病理：Pathology and biochemical pathology:1. 病理： 含色素的神經(jīng)元變性、缺失，尤以黑質(zhì)致密部DA能神經(jīng)元為著。出現(xiàn)路易小體,蒼白小體。 1. pathology: The loss and degeneration of pigmentosus neurons, especial the dopamine neurons in the substantia

13、 nigra. The lewy bodies and pale bodies can be seen also.,,,該二小體尚可在老年癡呆、共濟(jì)失調(diào)、毛細(xì)血管擴(kuò)張癥和哈-斯二氏病見到。 These two bodies also can be seen in senile dementia, ataxia, telangiectasia and Hallervoeden-spatz disease.,2.生化病理： PD 患者黑質(zhì)-紋狀體通路變性，紋狀體DA含量顯著降低（減少80%以上）， 2. biochemical pathology: Because the degeneration

14、 of substantia nigra- striatum pathway, the content of DA in striatum remarkably decrease (over 80 percent),,造成Ach系統(tǒng)功能相對亢進(jìn)，導(dǎo)致基底節(jié)輸出過多，丘腦-皮質(zhì)反饋活動受到過度抑制， the acetylcholine system exerts a excitatory effect, which results in the over output of basal ganglia, the inhibition of the feed backing activity of

15、 thalamus opticus-cortex,,其對皮質(zhì)的易化作用受到削弱，因此產(chǎn)生肌張力增高、動作減少等運(yùn)動癥狀。 and the impairment of facilitation effect to cortex, so the movement disorder symptoms appear, such as hypermyotonia, and reduction of movement,臨床表現(xiàn)：Clinical manifestation:1.大部分60歲以后發(fā)病。1. symptoms occur mostly in the age of 60, or older.2.

16、起病隱襲，緩慢發(fā)展，逐漸加劇。2. insidious onset, slow development, gradual aggravation,3.主要癥狀為靜止性震顫、肌張力增高、運(yùn)動遲緩等。首發(fā)癥狀：60-70%震顫，12%步行障礙，10%肌強(qiáng)直，10%運(yùn)動遲緩。 3. present symptoms are static tremor, hypermyotonia, bradykinesia. First symptoms: tremor 60-70%, walking disorder 12%, myotonia 10%, bradykinesia10%.,65-70%從一側(cè)上肢開

17、始，漸波及同側(cè)下肢，對側(cè)上肢與下肢。 25-30%從一側(cè)下肢開始。震顫: 靜止性，緊張加劇，搓丸樣動作These symptoms 65-70% start from one side upper extremity, gradually to same side lower extremity, then the opposite side extremities. Tremor: static, it increases with emotional stress, the fingers appear the pill-rolling- like action.,肌強(qiáng)直: 鉛管樣，齒輪樣

18、強(qiáng)直。路標(biāo)現(xiàn)象。Rigidity: lead pipe-like, cogwheel rigidity. A road sign phenomenon. 運(yùn)動障礙: 隨意動作減少，面具臉，寫字過小征。 motor disorder: voluntary action decreases, mask-like face, gradual smaller lettering,姿勢步態(tài)異常： 慌張步態(tài)等。 Posture and gait irregularity: such as the festinating gait. 其他：Myerson征，流口水，多汗，脂顏，便秘，認(rèn)知功能減退，抑郁，失眠

19、， Others: Myerson syndrome, dribbling, colliquative sweating, lipoids face, constipation, hypofunction of cognition, depression and insomnia,靜坐不能，疼痛，麻木，下肢浮腫，尿頻，視幻覺等。Acathisia, pain, numbness, edema of lower limbs, frequent micturition, optical illusion.,研究進(jìn)展 近來的進(jìn)展認(rèn)為帕金森病實質(zhì)上是涉及全腦的漸進(jìn)變性疾患，首先出現(xiàn)的是嗅覺的減退，

20、在黑質(zhì)的變性超過一定程度時出現(xiàn)帕金森癥狀，晚期出現(xiàn)抑郁、精神異常及癡呆等全腦功能的衰退。,輔助檢查：Auxiliary examination1.生化檢測：高效液相色譜示腦脊液與尿液中高香草酸、5-羥吲哚乙酸降低。1. biochemical detection: high performance liquid chromatogram shows increased homovanillic acid (HVA) and decreased 5-hydroxyindole acetic acid in cerebral spinal fluid and urine.,2.基因檢測：少數(shù)家族性

21、患者有基因突變。2. genic detection: gene mutations were found in a few familial patients.,3.功能顯象：PET 與SPECT可發(fā)現(xiàn)患者腦內(nèi) DAT功能顯著降低，且可早期發(fā)現(xiàn)， D2型DA 受體活性 3. Functional image: with PET and SPECT method, the DAT function in brain was detected to be significantly decreased in patients in early stage, the activity of D2

22、type receptor was,在疾病早期超敏，后期降低，以及DA遞質(zhì)合成減少。對PD的診斷與監(jiān)測均有價值。 supersensitive in early stage, then decreased with the decreased synthesis of DA transmitter. This phenomenon is valuable for the diagnosis and monitoring of PD.,診斷：Diagnosis根據(jù)年齡、臨床表現(xiàn)、病程發(fā)展。According to age, clinical manifestation, course of di

23、sease.,鑒別診斷： Differential diagnosis1.繼發(fā)性PD：均有明顯的病因可尋。1. Secondary PD: usually there are evident etiological factors（1）腦炎后帕金森綜合征：甲型腦炎后，罕見。乙型腦炎、愛滋病。 (1) Postencephalitic parkinsonism: post- encephalitis A, hardly seen. encephalitis B, AIDS.,（2） 藥物或中毒性帕金森綜合征：神經(jīng)安定劑（酚噻嗪類，丁酰苯類）、利血平、滅吐靈、甲基多巴、鋰、氟桂嗪、 (2) med

24、ical or toxical parkinsonism: neuroleptic (phenothiazines, butyrophenones ), reserpine, paspertin, methyldopa, lithium (Li), flunarizine,,腦益嗪。MPTP、錳、二硫化碳，一氧化碳中毒后遺癥等。 cinnarizine. The sequela of MPTP, manganese( Mn), carbon bisulfide, carbon monoxide poisoning.,（3） 動脈硬化性帕金森綜合征：病史、錐體束征、高血壓等可資鑒別。(3) a

25、rteriosclerosis parkinsonism: the differentiation depends on the medical record, pyramid sign, and hypertension.（4） 外傷性：病史可鑒。(4) Traumatic parkinsonism: according to the medical history.,2.伴發(fā)帕金森表現(xiàn)的其他神經(jīng)變性疾病（帕金森迭加綜合征）：The other neurodegenerative diseases accompanying parkinsonism (parkinsonism plus )（

26、1）進(jìn)行性核上性麻痹：垂直凝視不能，假性球麻痹，錐體束征，震顫不明顯，對多巴反應(yīng)差。 (1)Progressive supranuclear palsy: unable vertical steady fixation, supranuclear paralysis, pyramid sign, mild tremor, poor dopa reaction.,（2）多系統(tǒng)萎縮：(2) multiple system atrophy (MSA)*紋狀體黑質(zhì)變性：病理征、暈厥。striatonigral degeneration: pathological sign, faint*Shy-Dra

27、ger綜合征：直立性低血壓（差距40以上），性功能障礙，暈厥為主。Shy-Drager symptom： postural hypotension（difference over 40 mmHg ）, sexual disturbance, faint.,*橄欖腦橋小腦萎縮：小腦癥狀、錐體束征、脊髓癥狀。影象學(xué)可提供證據(jù)。Olive pons cerebellar atrophy: cerebellar symptom, pyramid sign, cord symptom.Evidences can be found with imageology(3) 帕金森綜合征-癡呆-ALS復(fù)合體：P

28、arkinsons syndrome-dementia-ALS(amyotrophic lateralizing sclerosis) complex,(4) 皮質(zhì)基底節(jié)變性：額頂葉限局性萎縮、氣球樣皮質(zhì)細(xì)胞肥大、黑質(zhì)色素脫失和 corticobasal degeneration（CBD）：fronto-parietal lobes focal atrophy; balloon-like cortic cells hypertrophy, pigment loss in substantia nigra and,廣泛的神經(jīng)元喪失?？捎兄w失用癥，多巴類藥及其激動劑有效。 General los

29、s of neurons. There may be limbs parectropia. Medication such as dopa alike and dopa- excitomotor will be effective,（5）偏側(cè)萎縮/偏側(cè)帕金森綜合征：病側(cè)出現(xiàn)癥狀，同側(cè)常有輕度萎縮。左旋多巴有效。 Hemiatrophy/hemiparkinsonism: hemilatera symptom and mild atrophy. Levodopa is effective.,3.遺傳性帕金森綜合征3. Heritage parkinsonism（1）常染色體顯性路易體?。涸缙诘?/p>

30、癡呆、幻覺、肌陣攣、步態(tài)障礙，對左旋多巴反應(yīng)差。 (1) Autosomal dominant lewy body disease: early dementia, illusion, myoclonus, gait disorder, poor reaction to levodopa.,（2） 肝豆?fàn)詈俗冃裕耗挲g小、肝損、角膜K-F環(huán)，血清銅、銅藍(lán)蛋白、銅氧化酶活性降低，尿銅增加。 (2) Kinnier-Wilson syndrome: young, liver injured, cornea K-F sign. serum copper and copper-protein are d

31、ecreased, and the activity of copper oxidase is decreased also. Urine copper is increased.,（3)亨廷頓舞蹈?。杭易迨?、癡呆、遺傳學(xué)檢查 (3) Humtingtons chorea: family history, dementia, test of genetics4. 抑郁癥：不具有肌強(qiáng)直與運(yùn)動遲緩，抗抑郁制劑有效。4. Depressive disorder: without myotonia and bradykinesia, anti-depressive medication is effe

32、ctive,5特發(fā)性震顫：震顫以姿勢性與運(yùn)動性，年齡早，飲酒或用心得安可緩解。無肌強(qiáng)直與運(yùn)動遲緩。1/3有家族史。5. Essential tremor: posture and motility tremor, young, relieving after drink or administrating propranolol, without myotonia and bradykinesia. 1/3 have family history.,治療：treatment:目標(biāo)：減輕癥狀、延緩進(jìn)程、提高生存質(zhì) 量。Goal: relieving symptom, postponing pr

33、ocess, enhancing living quality方法：依個體情況選擇治療（年齡、病情、藥物反應(yīng)等）Approach: The choice of therapies depends on the individual condition( age, state of illness, and the reaction to medication),1 神經(jīng)保護(hù)治療1. neuro-protection treatment （1） 單胺氧化酶抑制劑：B型單胺氧化酶抑制劑應(yīng)用較廣。常用丙炔苯丙胺（司來吉蘭），每次5毫克，1-2次/天，晨服。不良反應(yīng)：興奮、失眠、幻覺、妄想和胃腸不適。

34、 Monoamine oxidase inhibitor( MAOI): MAO-B is generally used. Selegiline frequently used, 5mg, 1-2 time per day morning. adverse effect:Exciting, insomnia, illusion, delusion and upset gastro-intestinal,（2） 其他：抗組胺藥、神經(jīng)營養(yǎng)因子、免疫調(diào)節(jié)劑、抗氧化劑、自由基清除劑等。 Others: antihistamine drug, neurotrophic factor, immunomod

35、ulator, antioxidant, free radical scavenger,2 非多巴胺能藥物2. Non-dopaminergic drugs （1） 抗膽堿能藥物：安坦：1-2毫克，每日3次。有膽堿能副作用，青光眼與前列腺肥大禁用。 Anticholinergic agents: antan, 1-2mg, Tid. Side effect: cholinergic effect, the uses in glaucoma and prostatic hypertrophy are forbidden,（2） 金剛烷胺：促進(jìn)DA合成與釋放，減少DA再吸收，早期患者可與安坦合用或

36、單用。副作用：浮腫、不寧。腎功能不全、癲癇、潰瘍、肝病慎用。哺乳期婦女禁用。0.1 2次/天Amantadine: facilitating the synthesis and releasing of DA, reducing the resorption of DA, it can be used in the early stage of disease combined antan or not. Side effect: swollen, restless. Cautious in renal inadequacy, epilepsy, ulcer and liver disease

37、. Women in lactation are forbidden.,（3）其他：抗抑郁藥治療抑郁癥，-受體阻滯劑治療姿勢性震顫，氯硝基安定治療痛性強(qiáng)直和構(gòu)音困難，氯氮平治療幻覺等精神癥狀。 Others: antidepressant for depression, - acceptor blocker for postural tremor, clonazepam for painful stiffness and dyslalia, clozapine for psychiatric symptom, such as illusion,3 多巴胺能藥物3. Dopaminergic d

38、rugs1）左旋多巴及復(fù)方左旋多巴：美多巴（加 卞絲肼，4：1），心寧美（信尼麥，加甲基多巴）。levodopa and benserazide : madopar (plus benserazide), sinemet(plus methyldopa)用藥時機(jī)：年老患者可早期選用。Administration opportunity: aged patients may be used in early stage of disease,,,用藥方法：由小到大Administration method: the dosage increases gradually副作用：周圍性為惡心、低血壓

39、，心律失常。中樞性為癥狀波動、運(yùn)動障礙、精神癥狀等。 Side effect: peripheral side effects: nausea, hypotension, arrhythmia. Central side effect: fluctuated symptom, motor disorder, psychiatric symptom, etc.,禁用：前列腺肥大、窄角型青光眼、嚴(yán)重肝腎功能不全。Forbidden: prostatic hypertrophy, narrow angle glaucoma, severe liver and renalInadequacy.長期及大

40、劑量使用可出現(xiàn)：long-term and large dosage will cause:,*癥狀波動：因藥物作用時間縮短和血藥濃度不穩(wěn)定，出現(xiàn)劑末運(yùn)動不能和雙向運(yùn)動障礙。 fluctuated symptom: because of the reduction of drug reaction time, and the astable drug concentration in blood, the end of dose akinesis and bidirection motor disorder appear.,開-關(guān)（on-off）現(xiàn)象。低張力凍結(jié)現(xiàn)象與藥物慢性中毒和病情加重有關(guān)

41、，改變用藥途徑、方法、劑型及阿撲嗎啡有時可緩解癥狀。 on-off phenomenon. low tension freezing phenomenon is related to chronic intoxication and the aggravated disease. Change in the way of administration, dose, dosage form and giving apomorphine some times will relieve these symptoms.,*異動癥：舞蹈樣，手足徐動樣或肌陣攣性運(yùn)動異常，可波及局部或全身。與紋狀體受體的超

42、敏感有關(guān)，減藥或給泰必利有效。 Abnormal involuntary movement: dancing-like, athetosis-like, or myoclonic abnormal movement involve local or all over the body. This phenomenon is related to the supersensitivity of receptor in striatum, reducing dose or giving tiapride will be effective.,2） DA受體激動劑：溴隱亭、培高利特（協(xié)良行,因為其有

43、引起心臟瓣膜與肺纖維化的副作用，2008年我國停用，替代藥品為森福羅，是新一代非麥角類多巴胺受體激動劑，化學(xué)名為普拉克索。 為非麥角堿類D2和D3受體激動劑 ）。DA receptor agonist: Bromocriptine, pergolide3）兒茶酚-氧位-甲基轉(zhuǎn)移酶抑制劑：托卡朋等。Catechol-o- methyltransferases: Tolcapone, etc.,4 外科治療：4. Surgical therapy:1）重建性手術(shù)：體內(nèi)移植多巴胺能神經(jīng)元。正在研究之中。 Reconstructive procedure: dopaminergic neuron tr

44、ansplantation in vivo.2）破壞性手術(shù)：蒼白球毀損術(shù)、丘腦毀損術(shù)、深部腦刺激Destructive procedure: globus pallidus lesion, thalamus lesion, deep brain stimulation,預(yù)后：prognosis:慢性發(fā)展（也有快發(fā)展的），無根治方法，多死于并發(fā)癥（肺炎、骨折）。Usually this disease aggravates slowly( but there are fast develop case), can not be cured completely, most die of comp

45、lication(pneumonia, bone fracture),小舞蹈病 chorea minor,小舞蹈病又稱風(fēng)濕性舞蹈病，Sydenham舞蹈病, 是風(fēng)濕熱在神經(jīng)系統(tǒng)的表現(xiàn)。Chorea minor also named rheumatic chorea, sydenham chorea, it is the manifestation of rheumatic fever in nervous system.,病因及病機(jī)Etiological factor and pathogenesis與A族溶血性鏈球菌感染有關(guān)?；颊哐逯锌刹榈娇股窠?jīng)元抗體。Onset is related

46、to the A group hemolytic streptococcal infection, the anti-neuron antibody can be found in the patients serum.,病理pathology黑質(zhì)、紋狀體、丘腦底部、小腦齒狀核、大腦皮質(zhì)可逆性炎癥性改變。90%尸解可發(fā)現(xiàn)有風(fēng)濕性心臟病的證據(jù)。Reversible inflammatory changes appear in substantia nigra, striatum,subthalamus, dentate body of cerebellum, cortex. 90% necros

47、copy can find evidences for rheumatic heart disease.,臨床表現(xiàn)Clinical manifestationa) 約80%患者為5-15歲，女多于男，約1.5：1到3.2：1。About 80% patients are around the age of 5-15 years, female more than male, about 1.5: 1 to 3.2: 1.,b)多為亞急性或隱襲發(fā)病。1/3病例出現(xiàn)舞蹈癥狀前2-6個月或更長時間內(nèi)有溶血性鏈球菌感染史。 most are subacute or smoldering onset.

48、1/3 patients had the history ofhemolytic streptococcal infection 2-6 months or longer before those chorea symptoms appear.,c)早期精神癥狀：激動、易激惹、注意力散漫、學(xué)業(yè)退步，行為改變。 Psychiatric symptom in early stage: agitating, irritable, difficult to focus attention, school work retrogression, behavior change,d) 早期運(yùn)動癥狀：動作不

49、協(xié)調(diào)、字跡歪斜、持物不穩(wěn)、行走搖晃。 Motor symptoms in early stage: action out of step, askew handwriting, unsteadiness in hold, shaky walking.,a)舞蹈樣動作：常雙側(cè)，約20%為偏側(cè)或局限。面部最明顯，擠眉弄眼、噘嘴、吐舌、鬼臉。 Chorea-like action: usually bilateral, about 20% are one side or focal. Most in face, tapir mouth, tongue protrusion, grimace.,肢體出

50、現(xiàn)極快的、不規(guī)則的、無目的的、不自主運(yùn)動，變幻莫測。 Extremities appear fast, irregular, purposeless involuntary movement in an unpredictable way.,上肢比下肢明顯，情緒緊張時加重，安靜減輕，睡時消失。常在2-4周內(nèi)加重，3-6個月緩解。Upper limb is more obvious than lower limb, aggravation in emotional stress, relief at rest, disappearing at bedtime. Usually aggravati

51、ng during 2-4 weeks, relieving after 3-6 months.,b)肌張力及肌力減退：肌張力及肌力的減退使腕、掌、指關(guān)節(jié)過伸，稱舞蹈病手姿。 Muscular tension and muscle strength decrease: hyperextension appear at the joints of wrist, palm, and finger, called chorea gesture.,因手指的小幅舞動可在握手時感覺出患者的手時緊時松，稱擠奶婦手法，或盈虧征。 Because the finger flexion-extension, ex

52、aminer can find patients finger flexion-extension chorea resulting in the characteristic oscillating milkmaid grip, or wax-waning sign.,肌張力及肌力減退與舞蹈樣動作、共濟(jì)失調(diào)一起構(gòu)成小舞蹈病三聯(lián)征。 muscular tension and muscle strength decrease, chorea-like action, and ataxia constitute the chorea minor triad.,c)精神癥狀：躁動不安、精神錯亂、幻覺

53、、妄想，稱躁狂性舞蹈病。Psychiatric symptom: restlessness, mental disorder, illusion, delusion, called chorea insaniens.,d)其他風(fēng)濕病表現(xiàn)：發(fā)熱、風(fēng)濕性關(guān)節(jié)炎、風(fēng)濕性心臟炎、皮下結(jié)節(jié)、血沉加快、抗“O”滴度增加。Othe rheumatic disorder features: fever, rheumatic arthritis, rheumatic heart disease, subcutaneous nodule, raised blood sedimentation, raised AS

54、O titre .,輔助檢查auxiliary examination1.血清學(xué)檢查：白細(xì)胞增加、血沉增快、粘蛋白增多、C反應(yīng)蛋白效價提高、抗“O”滴度增加。Serologic examination: raised leucocyte, raised blood sedimentation, raised mucoprotein, raised ASO titre .,2.咽拭子培養(yǎng)：A組溶血性鏈球菌。Pharynx swab culture: A group hemolytic streptococcus.3.腦電圖：無特異性。輕度彌漫性慢波活動。EEG(electroencephalog

55、ram): non-specificity,4.影象學(xué)：CT可見尾狀核區(qū)低密度灶及水腫，MRI可見尾狀核、殼核、蒼白球增大，T2高信號。PET可見紋狀體呈高代謝改變，可恢復(fù)。Screenage: low density focus and edema can be seen in caudate nucleus area with CT scan, The swelling and T2 high signal can be seen in caudate, putamen, and globus pallidus with MRI. The Hypermetabolism in striat

56、um can be seen with PET, and it is retrievable.,5.肌電圖：持續(xù)大于100毫秒的電活動發(fā)放。EMG: persistent electric activity releasing over 100 milliscond,診斷及鑒別診斷Diagnosis and differential diagnosis診斷依據(jù)年齡、特征性舞蹈病三聯(lián)征、風(fēng)濕病的其他表現(xiàn)。 According to age, marked chorea triad and the other features of rheumatic disease,鑒別診斷Differenti

57、al diagnosis1.習(xí)慣性痙攣：多見在兒童，特點是刻板的動作重復(fù)，常局限在同一肌肉或肌群；無肌力、肌張力及共濟(jì)運(yùn)動的異常。 Habit spasm: mainly in children, stereotyped repeating movement, usually is limited in same muscle or muscles, without the abnormal muscle power ,muscular tension and ataxia.,2.先天性舞蹈?。憾?歲前發(fā)病，為腦癱的一種表現(xiàn)，常伴有智能障礙、震顫和痙攣性癱瘓。 Congenital chore

58、a: most onset before two years old, a feature of cerebral palsy with disturbance of intelligence, tremor, and spastic paralysis,3.抽動穢語綜合征：見于兒童，快速刻板的肌肉運(yùn)動，常累及頭面、頸咽部肌肉。還有怪聲與臟話。 Gilles de la Tourette syndrome: mainly attack children, fast and stereotyped muscular movement, usually involving the muscles

59、of head , face, neck and pharyngeal portion. Strange voice and bad language.,4.亨廷頓舞蹈病：中年以上，有遺傳史與癡呆。Humtingtons chorea: over middle-aged, family history and dementia.,5.扭轉(zhuǎn)痙攣：也常見兒童，扭轉(zhuǎn)動作較快，常持續(xù)存在，沒有自限性，肌張力在扭轉(zhuǎn)時升高。 Torsion spasm: children, torsion movement is fast and persisting with increased muscular te

60、nsion. Uncontrollable torsion movement.,6.肝豆?fàn)詈俗冃裕?Kinnier-Wilson syndrome7.高或低血鈣癥：Hypercalcemia or hypocalcemia8.抗心磷脂抗體綜合征： Anticardiolipin syndrome,治療treatment 1. 一般處理：臥床休息，安靜，低亮度，防止刺激與外傷。注意營養(yǎng)。General handling: bed rest, quiet, low brightness, avoiding stimulation and injury. Good nutrition.,2. 病因治

61、療：青霉素或其他有效抗菌素，10-14天。同時給水楊酸或強(qiáng)的松，癥狀消失后漸減量，停藥。Etilogical treatment: penicillin or other effective antibiotic, 10-14 days, with salicylic acid or prednisone, reducing the dose gradually, than withdrawal after the symptoms disappearing.,3.對癥治療：安定5毫克，或硝基安定7.5毫克每日2-3次口服；氯丙嗪12.5-25毫克，每日2-3次；氟哌啶醇0.5-1毫克，每日2

62、-3次。后兩種藥需注意錐體外系副反應(yīng)。 Symptomatic treatment: Diazepam 5mg, or Nitrazepam 7.5mg bid , tid;Chlorpromazine 12.5-25mg, bid, tid; Haloperidol0.5-1mg, bid, tid. The side effect of latter two drugs is extrapyramidal symptoms.,4.有明顯風(fēng)濕熱表現(xiàn)者要給糖皮質(zhì)激素。Glucocorticoid should be given while patients appear apparent features of rheumatic fever.,預(yù)后prognosis 為自限性，3-6個月自行緩解；治療可縮短病程，1/4可復(fù)發(fā)，1/5死于心臟并發(fā)癥。To be self limited, relieving in 3-6 months; proper treatment may shorten the course of disease, 1/4 may recur, 1/5 may die of cardiac complication.,Thank you !,