运动障碍病-中国协和医科大学

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1、运动障碍病中国协和医科大学北京协和医院神经科万新华运动障碍病的定义锥体外系疾病：Extrapyramidaldiseases由于脑基底节或黑质结构功能紊乱导致的以随意运动调节功能障碍为特征的一组疾病运动障碍疾病：MovementDisorders发生于意识清醒病人的随意运动调节功能障碍为特征的一组疾病运动障碍病的特点通常与基底节或多巴胺神经递质传导功能紊乱有关；临床上导致运动调节功能障碍，而非运动能力本身；故肌力、感觉和小脑一般不受影响；临床上主要表现为：肌张力降低-运动过多所致的异常不自主运动和肌张力增高-运动减少所致的运动贫乏两大类；所有不自主运动受病人精神状态影响，紧张时加

2、重，睡眠时消失。运动障碍病分类运动增多类腹部异动症(Abdominaldyskinesias)静坐不能性运动(Akathiticmovements)协调不能/共济失调(Asynergia/ataxia)手足徐动症(Athetosis)投掷症(Ballism)舞蹈症(Chorea)辩距不良(Dysmetria)肌张力障碍(Dystonia)运动增多类偏侧面肌痉挛(Hemifacialspasm)惊跳症（Hyperekplexia〕睡眠源性异动症(Hypnogenicdyskinesias)肢残跳动（Jumpystumps〕痛肢趾（指）多动症（Painfullegs-movingto

3、es/fingerssyndrome）肌阵挛(Myoclonus)运动增多类肌蠕动（Myokymia）myorhythmia（Whipple’sdisease）发作性异动症（Paroxysmaldyskinesias,PKD和PNKD）不安腿(Restlesslegs)刻板症(Stereotypy)抽动症(Tics)震颤(Tremor)运动减少类运动不能/运动迟缓（Akinesia/bradykinesia,parkinsonism)失用(Apraxia)阻滞性抽动症（Blockingtics）紧张症，精神运动性抑郁和强迫症性迟缓(Catatonia,psychomotordep

4、ression,andobsessionalslowness)冻结现象(Freezingphenomenon)甲状腺功能低下性迟缓(Hypothyroidslowness)僵人综合症(Stiff-personsyndrome）THEPREVALENCEOFMDsENCOUNTEREDINMDCLINICSMOVEMENTDISORDERNUMBEROFPATIENTSPERCENTParkinsonism7,56432.9Parkinson’sdisease5,410Progressivesupranuclearpalsy381Multiplesystematrophy349Co

5、rtical-basalganglionicdegeneration143Vascular265Drug-induced227Hemiparkinsonism-hemiatrophy81Gaitdisorder187Other521THEPREVALENCEOFMDsENCOUNTEREDINMDCLINICSMOVEMENTDISORDERNUMBEROFPATIENTSPERCENTDystonia6,79831.3Primarydystonia5,247focal61%segmental30%generalized9%Secondarydystonia1,551Hemidy

6、stonia191Tardivedystonia472Other888THEPREVALENCEOFMDsENCOUNTEREDINMDCLINICSMOVEMENTDISORDERNUMBEROFPATIENTSPERCENTTremor3,01313.9Essentialtremor2,082Cerebellar151Midbrain(‘rubral’)86Primarywriting59Orthostatic16Other619THEPREVALENCEOFMDsENCOUNTEREDINMDCLINICSMOVEMENTDISORDERNUMBEROFPATIENTSPE

7、RCENTTics(Tourette’ssyndrome)1,0224.7Chorea6583.1Huntington’sdisease282Hemiballism62Other314Tardivesyndromes5832.7Myoclonus5472.5MOVEMENTDISORDERNUMBEROFPATIENTSPERCENTHemifacialspasm3591.7Ataxia3161.5Paroxysmaldyskinesias1690.8Stereotypies(o