2008年12月FDA批准了Prestwick公司用于治疗亨廷顿舞蹈病的药物Xenazine（丁苯那嗪），这是首个在美国获准治疗该症的治疗药。它获准的依据是一项III期临床实验结果显示，这种具有选择性作用功能的多巴胺耗竭治疗药疗效显著，并且安全性和耐受性良好。 Prestwick表示丁苯那嗪将在一项经FDA批准的风险评估项目下进行销售，这个评估项目主要防止该药有可能导致的抑郁和自杀倾向等不良反应。此前，该药也已被指定为罕见病治疗药，它在美国具有7年的独家销售权。该药获准后在美国受到欢迎，当地约有3万名患者深受这种疾病的困扰。美国遗传性疾病基金会表示，舞蹈病不仅给患者带来不便，它还会严重影响患者在步行、说话、工作和看电视等各方面的能力。目前，该药已在其他14个国家通过批准，新近获准的国家包括荷兰、瑞典和瑞士。 英文药名:Xenazine(Tetrabenazine Tablets) 中文药名: 丁苯那嗪片 生产厂家: Not available 药品介绍 这个药通常也叫: Nitoman 药品名称：丁苯那嗪片 主要成分：丁苯那嗪  药理毒理 丁苯那嗪通过耗竭大脑神经末梢内的多巴胺来发挥作用，主要用于控制运动功能障碍。 药代动力学 吸收:口服后经胃肠道吸收差，且不稳定。代谢:大部分经肝脏首过代谢作用还原为羟基丁苯那嗪。排泄:以代谢物形式经尿液排泄。 适应症 治疗舞蹈病、投掷症以及其它运动机能亢进性疾病。 用法用量 口服给药。服药不受进食影响。 成人:初始量：12.5mg，每天2次，逐渐增加至12.5-25mg，每天3次。如果最大剂量用药7天仍病情没有改善，则用该药可无效。 老人:初始量：12.5mg/天，然后逐渐增加剂量。 中重度迟缓性运动障碍：成人:初始量：12.5mg/天，根据反应增加剂量。 任何疑问，请遵医嘱！ 药物过量 症状：困倦，出汗，低血压，低体温。 处理：支持和对症治疗。 禁忌 对本品过敏者禁用；禁用于哺乳期妇女。 注意事项 可加重帕金森病的症状。影响驾车和机械操作能力。妊娠期妇女慎用。 不良反应 锥体外系症状、帕金森综合征，极少数出现急性肌张力异常，其它常见的不良反应包括困倦、疲劳、紧张、焦虑、失眠、兴奋、精神混乱、流涎、体位性低血压、恶心、头晕、偏执、皮疹、性欲减退、阳痿。 严重不良反应:抗神经病药恶性综合征。 药品相互作用 在单胺氧化酶抑制剂（MAOI）治疗期间或停药后14天内，不能给予本药。本药可阻断利血平的作用。本药消弱左旋多巴的作用，导致帕金森病加重。本药与金刚烷胺、甲氧氯普胺或抗精神病药合用，增加发生椎体外系副作用的风险。 FDA妊娠分级：C级:动物研究证明药物对胎儿有危害性（致畸或胚胎死亡等），或尚无设对照的妊娠妇女研究，或尚未对妊娠妇女及动物进行研究。本类药物只有在权衡对孕妇的益处大于对胎儿的危害之后，方可使用。 XENAZINE®: 25 mg, scored tablets (INN: tetrabenazine) Xenazine® is indicated for the treatment of Huntington’s chorea. GENERIC NAME: Tetrabenazine BRAND NAME: Xenazine How does it work? Xenazine (Tetrabenazine) works by depleting the stores of certain chemicals called neurotransmitters within nerve cells in the brain. These neurotransmitters include dopamine, serotonin and noradrenaline and are normally involved with the passage of messages between nerve cells in the brain and the normal functioning of the brain and nervous system. Depleting the brain of these neurotransmitters can help control movement disorders caused by diseases of the brain and nervous system, although the exact mechanism of action is not fully understood. Xenazine (Tetrabenazine) is mainly used to control movement disorders associated with diseases of the brain and nervous system, such as Huntington's chorea, but can also be used to control movement disorders caused by antipsychotic medicines. The older antipsychotic medicines such as chlorpromazine, haloperidol and flupentixol can cause side effects involving involuntary movement of the tongue, face, mouth and jaw, which may sometimes be accompanied by involuntary movements of the arms and legs (tardive dyskinesia). If this is disabling or socially embarrasing, and persists despite stopping or reducing the dose of the antipsychotic, the symptoms can be treated using tetrabenazine. Xenazine (Tetrabenazine) can also be used if it is not possible to stop or change the antipsychotic. What is it used for? Movement disorders associated with disease of the brain and spinal cord Nerve cell breakdown resulting in irregular involuntary movements and brain disease (Huntington's chorea) Rhythmical involuntary movement of the tongue, face, mouth and jaw, which may sometimes be accompanied by involuntary movements of the arms and legs (tardive dyskinesia)Warning! Do not drink alcohol whilst taking this medicine. This medicine may cause drowsiness. If affected do not drive or operate machinery. Not to be used in: No known conditions This medicine should not be used if you are allergic to one or any of its ingredients. Please inform your doctor or pharmacist if you have previously experienced such an allergy. If you feel you have experienced an allergic reaction, stop using this medicine and inform your doctor or pharmacist immediately. Pregnancy and Breastfeeding: Certain medicines should not be used during pregnancy or breastfeeding. However, other medicines may be safely used in pregnancy or breastfeeding providing the benefits to the mother outweigh the risks to the unborn baby. Always inform your doctor if you are pregnant or planning a pregnancy, before using any medicine. Ther is no information about the safety of this medicine during pregnancy, but it has been widely used for many years without apparent ill consequence. As there is no safety information available, the manufacturer does not recommend the use of this medicine when breastfeeding. Alternatively, if this medicine must be used, the mother should not breastfeed. Seek medical advice from your doctor. Label warnings: This medication may cause drowsiness. If affected do not drive or operate machinery. Avoid alcoholic drink. Side effects: Medicines and their possible side effects can affect individual people in different ways. The following are some of the side effects that are known to be associated with this medicine. Because a side effect is stated here, it does not mean that all people using this medicine will experience that or any side effect. Disturbances of the gut such as diarrhoea, constipation, nausea, vomiting or abdominal pain Low blood pressure (hypotension)High temperature combined with falling levels of consciousness, paleness, sweating and a fast heart beat (neuroleptic malignant syndrome). Requires stopping the medicine and immediate medical treatment. The side effects listed above may not include all of the side effects reported by the drug's manufacturer. For more information about any other possible risks associated with this medicine, please read the information provided with the medicine or consult your doctor or pharmacist. How can this medicine affect other medicines? Xenazine (Tetrabenazine) should not be taken at the same time as, or for 14 days following a course of monoamine oxidase inhibitor (MAOI) antidepressants, as this can cause restlessness, disorientation and confusion. Xenazine (Tetrabenazine) opposes the effects of levodopa. Xenazine (Tetrabenazine) should not be taken with alcohol as this may increase the drowsiness experienced. Indications for XENAZINEHuntington's chorea. Adult dose for XENAZINEIndividualize. Initially 12.5mg once daily in the AM, then 12.5mg twice daily after one week. Titrate slowly at weekly intervals by 12.5mg. Doses of 37.5–50mg/day should be given in a 3 times/day regimen, max 25mg/dose. Patients who require ≥50mg/day should be genotyped for CYP2D6. Poor metabolizers (CYP2D6): max 25mg/dose and 50mg/day; extensive and intermediate metabolizers: max 37.5mg/dose and 100mg/day. Concomitant strong CYP2D6 inhibitors (eg, paroxetine, fluoxetine, quinidine): reduce tetrabenazine dose by ½; moderate to weak CYP2D6 inhibitors: not evaluated. Retitrate if therapy interrupted for more than 5 days. Children's dosing for XENAZINENot recommended. Contraindications for XENAZINEDepression. Suicidal ideation. Hepatic impairment. Concomitant monoamine oxidase inhibitors (MAOIs). During or within 20 days of stopping reserpine. Precautions for XENAZINEAvoid in congenital long QT syndrome or history of cardiac arrhythmias. Bradycardia. Hypokalemia. Hypomagnesemia. Recent MI. Unstable heart disease. Cardiovascular disease. History of depression, suicidal ideation, or breast cancer. Monitor for depression, emotional lability, akathisia. Poor metabolizers (CYP2D6). Reevaluate periodically. Pregnancy (Cat.C). Nursing mothers: not recommended. Interactions for XENAZINESee Contraindications. Avoid other drugs that can cause QT prolongation (eg, chlorpromazine, thioridazine, ziprasidone, moxifloxacin, quinidine, procainamide, amiodarone, sotalol). Potentiated by CYP2D6 inhibitors (eg, paroxetine, fluoxetine). Increased risk of neuroleptic malignant syndrome, extrapyramidal syndrome with neuroleptics, dopamine antagonists. Additive CNS depression with alcohol, other CNS depressants. Adverse Reactions for XENAZINESedation, fatigue, insomnia, depression, dysphagia, akathisia, nausea, parkinsonism, QTc prolongation, orthostatic hypotension, elevated serum prolactin; rarely: neuroleptic malignant syndrome, tardive dyskinesia, extrapyramidal effects. How is XENAZINE supplied?Tabs—112 Related Disease:Huntington's disease 什么是风湿性舞蹈病(小舞蹈病) 风湿性舞蹈病又称小舞蹈病或Sydenham舞蹈病是与风湿密切相关的临床以不规则舞蹈样不自主动作和肌张力降低为主要表现的一种弥散性脑病。 什么原因引起风湿性舞蹈病(小舞蹈病) 本病与风湿病密切相关，常为急性风湿病的一种表现，孕妇发生的妊娠性舞蹈病也多为风湿性舞蹈病的复发。也可见于猩红热、白喉等其它感染性疾病之后。 风湿性舞蹈病(小舞蹈病)有什么症状 本病多发生在5～15岁的儿童或少年，女性居多。通常呈亚急性起病，早期常有不安宁、易激动、注意力不集中等表现，随着不自主运动的日趋明显而引起注意。 一、神经系统损害症状： （一）底节症状：表现为突发、急促、不规则、无目的的舞蹈样不自主动作。常起自一侧肢体，然后波及对侧，有肢症状多较下肢重，偶也可限于一侧，不时的出现手指屈伸，翻举旋臂、踢腿屈膝等动作。面肌的不自主动作可见挤眉弄眼、张口吐舌等，犹如作鬼脸。躯干部肌肉可出现突肤挺胸、脊背歪扭等动作。倘影响吞咽、构音及呼吸肌时可致吞咽、构音障碍及呼吸不规则。以上不自主动作在情绪激动时加剧、安静时减轻、睡眠时消失。严重者几乎整日不停，影响日常生活和无法行立坐卧。 （二）大脑皮层症状：情绪不稳、易激动，常影响睡眠和休息，严重者可有精神错乱和躁动等。 （三）小脑症状：肌张力明显降低，腱反射减弱甚至消失，动作苯拙，共济失调。 二、全身症状： 多数患者在病前或有的在病程中有发热、咽痛、扁桃腺炎、关节疼痛等见风湿症状，心脏受累时可有心率增快、心脏扩大和杂音。 风湿性舞蹈病(小舞蹈病)需要做哪些检查 1、化验可有血沉增快，抗O增高，C反应蛋白阳性，血液某些免疫功能指标异常等阳性发现。 2、脑脊液压力和成分大多正常。 3、脑电图半数患者显示异常，但多轻微。 如何治疗 患者应卧床休息，避免声、光等环境因素的刺激，保护因不自主动作可能带来的意外损伤，进富含营养及易消化的饮食。 一、病因治疗：针对风湿可应用阿斯匹林口服，如疗效欠佳可加用强的松或地塞米松，当症状控制后可逐渐减量乃至停药。如有发热、咽痛等症状时应加用青霉素。 二、对症治疗：可选用鲁米那，安定或硝基安定。 本病预后良好，多在2～3月内完全恢复。少数可遗留一些轻微神经体征，如突发的随意动作、动作不协调等。约1/3患者可有复发。 --------------------------------------------------------------- 注：以下产品不同规格和不同价格，购买时请以电话咨询为准！ --------------------------------------------------------------- 产地国家: 加拿大 原产地英文商品名: Xenazine 25mg/tab 112tabs/bottle 原产地英文药品名: tetrabenazine 中文参考商品译名: Xenazine 25毫克/片 112片/瓶 中文参考药品译名: 丁苯那嗪 生产厂家中文参考译名: Cambridge Laboratories 生产厂家英文名: Cambridge Laboratories --------------------------------------------------------------- 产地国家: 加拿大 原产地英文商品名: Generic Xenazine 25mg/tab 100tabs/bottle 原产地英文药品名: tetrabenazine 中文参考商品译名: Xenazine仿制药 25毫克/片 100片/瓶 中文参考药品译名: 丁苯那嗪