服藥亨廷頓舞蹈病

Even though there is currently no therapy available to delay the onset of Huntington Disease (HD) symptoms or prevent its progression, there is a symptomatic treatment for patients, that may assist in improving the quality of life and prevent complications. Similar to other neurological diseases, HD place individuals at higher risk of experiences side effects from medication, particularly cognitive adverse effects. It is important to avoid polypharmacy if possible. Symptomatic treatment for HD can be segmented into drugs to treat the movement disorder medications that are for psychiatric or behavioral problems.

什麼是亨廷頓病 (HD)

There are experimental therapies for HD that are being tested in animal models as well as human trials. Gaining knowledge of ongoing research, to find a cure for HD, should be part of the care plan of the individual patient as well as their family.

Therapeutic options include dopamine-depleting agents such as reserpine tetrabenazine and dopamine-receptor antagonist (例如, 抗精神病藥). 當使用這些藥物的長期, 有不良副作用的額外風險. 如果與HD患者有舞蹈病運動, 這應該是唯一的藥理學治療,如果變成禁止給病人. 安定藥可引起變性疾病的其他功能, 如剛性, 和運動遲緩, 造成進一步的功能惡化.

一些研究的結果已暗示丙戊酸和氯硝西泮,可有效地舞蹈病的治療, while results from other studies have been less conclusive. In many doctor’s experiences using valproic acid and clonazepam initially may be beneficial evaluating because of their safer adverse-effect profiles.

Tetrabenazine is a dopamine-depleting agent was authorized by the FDA in August of 2008. It may be more effective than reserpine in the treatment of chorea and less likely to cause hypotension. The dose is titrated gradually and may be rising over several weeks to a maximum 75-100 mg/d in divided doses.

Using Monoamine Inhibitors

Drug Class Summary
Antichorea effects of the central monoamine-depleting agent are thought to be associated with its effect on reversible depletion of monoamines from nerve terminals, as known as either serotonin, dopamine or norepinephrine.

丁苯 ( Xenazine)
Deepelets neurotransmitter stores of noradrenaline, 血清素, and dopamine within nerve cells in the brain, thus adjusting the transmission of electric signals from the brain that control movement by reversibility hindering vesicular monoamine transporter 2 (VMAT2).

Efficacy and safety confirmed in a randomized, 雙盲, placebo-controlled, multi-location study. Patients treated with tetrabenazine saw meaningful improvement in chorea compared to patients treated with the placebo. There is additional research supporting this conclusion. Indicated for chorea linked with Huntington disease.

Deutetrabenazine (Austedo)

Drug Class Summary

This drug is administered via the mouth, VMAT-2 inhibitor is shown as a treatment for chorea associated with Huntington disease.
Reserpine

Dopamine-depleting agent. 在過去使用來治療高血壓.

Anticonvulsant

Drug Class Summary
這些試劑用於控制在舞蹈病肌肉痙攣.
丙戊酸 (RX, Depakene, Depacon)

羧酸通常用作抗癲癇藥, 情緒穩定劑在躁狂症, 和用於偏頭痛預防藥. 當與丙戊酸鈉在混合 1:1 摩爾關係, 地稱為雙丙戊酸鈉. 由丙戊酸行使其抗癲癇作用的設備尚未確認; 其活性可以鏈接到GABA的提高腦水平. 沒有大型臨床試驗存在支持其過動症的使用, 但它可能是有用的, 如患者所暗示的幾個小研究,不同病因的舞蹈症.
的每日最大劑量 2000 毫克以分開的劑量 (出價或TID) 足以確定藥物是否將是有效的對個體患者.

氯硝西泮 (克諾)

Drug Class Summary

氯硝西泮屬於苯二氮卓類藥物, 這增強GABA的活性, 在中樞神經系統的主要抑制性神經遞質.
經常使用的抗癲癇藥. May be beneficial in the treatment of chorea, but no large clinical trials exist to support its use. Does not induce parkinsonism or carry the risk of tardive syndromes, as neuroleptics do; 因此, an adequate trial of this medication is reasonable before using dopamine antagonists.
Maximum daily dose of 2-4 mg divided bid/tid usually is enough to determine effectiveness for the individual patient.

Antipsychotic agents
Drug Class Summary

These agents may enhance choreic movements in patients.
Risperidone (維思通)

An antipsychotic agent that belongs to a newer chemical class called benzisoxazole derivatives.
This is considered antagonist of type 2 dopamine and serotonin receptors.
Less likely than typical neuroleptics to cause parkinsonism.
Haloperidol (氟哌啶醇)

This is the initial or first of the butyrophenone class of major tranquilizers. Typical neuroleptics, 例如氟哌啶醇, are potent dopamine-receptor antagonists and are recommended to be used only as a physician’s last recourse to treat chorea.

抗抑鬱藥

Drug Class Summary
Depression is relatively common in patients with HD and should be treated pharmacologically as quickly as the diagnosis of depression is given. Depression in subjects with HD can be administered the same agents used for the treatment of depression of any other cause. SSRIs can be applied as first-line therapy since they have a low adverse-effect profile, convenient dosing, and safety in the event of an overdose. Other antidepressants can be used, including bupropion, nefazodone, venlafaxine, and the tricyclic antidepressants. Electroconvulsive therapy can be effective if a quick intervention is needed and in patients who do not respond to numerous trial-effective medications.

帕羅西汀 (帕羅西汀)
Paxil is SSRI that can be used once daily. Most patients should take it in the morning because can be stimulating and may cause insomnia. If sedation occurs, the drug should be taken at bedtime. A few patients develop sexual problems, such as decreased libido, anorgasmia, or ejaculatory delay.