Medicin til behandling af Huntingtons sygdom

Selv om der er i øjeblikket ingen behandling til rådighed til at sinke udviklingen af ​​Huntingtons sygdom (HD) symptomer eller forhindre dens progression, der er en symptomatisk behandling af patienter, der kan bidrage til at forbedre livskvaliteten og forebygge komplikationer. I lighed med andre neurologiske sygdomme, HD sted individer med højere risiko for erfaringer bivirkninger fra medicin, især kognitive bivirkninger. Det er vigtigt at undgå polyfarmaci hvis det er muligt. Symptomatic treatment for HD can be segmented into drugs to treat the movement disorder medications that are for psychiatric or behavioral problems.

What is Huntington’s Disease (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 (e.g., neuroleptics). When using these drugs long term, there is the additional risk of adverse side effects. If a patient with HD has Choreic movements, this should be treated pharmacologically only if it becomes disabling to the patient. Neuroleptics may cause degeneration in other features of the disease, such as rigidity, and bradykinesia, causing further functional deterioration.

Resultaterne af nogle undersøgelser har antydet, at valproinsyre og clonazepam kan være effektive i behandling af chorea, mens resultater fra andre undersøgelser har været mindre overbevisende. I mange lægens erfaringer ved hjælp af valproat og clonazepam i første omgang kan være gavnligt at evaluere på grund af deres mere sikre adverse effect profiler.

Tetrabenazin er en dopamin-nedbrydende middel blev godkendt af FDA i august 2008. Det kan være mere effektiv end reserpin i behandling af chorea og mindre tilbøjelige til at forårsage 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.

Tetrabenazine ( Xenazine)
Deepelets neurotransmitter stores of noradrenaline, serotonin, 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, dobbeltblindede, 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.

Dopamine-depleting agent. Used in the past to treat hypertension.


Drug Class Summary
These agents are employed to control muscle spasms in chorea.
Valproic acid (rx, Depakene, Depacon)

Carboxylic acid commonly used as an antiepileptic drug, mood stabilizer in mania, and prophylactic agent for a migraine. When mixed with sodium valproate in 1:1 molar relation, termed divalproex sodium. The device by which valproate exercises its antiepileptic effects has not been confirmed; its activity may be linked to heightened brain levels of GABA. No large clinical trials exist to support its use for hyperkinetic movement disorders, but it may be useful, as implied by a few small studies in patients with chorea of different etiologies.
The maximum daily dose of 2000 mg in divided doses (bid or tid) is enough to determine whether the drug is going to be effective for the individual patient.

Clonazepam (Klonopin)

Drug Class Summary

Clonazepam belongs to benzodiazepine class of drugs, which enhances the activity of GABA, a major inhibitory neurotransmitter in CNS.
Used regularly as an antiepileptic drug. 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; therefore, 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 (Risperdal)

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 (Haldol)

This is the initial or first of the butyrophenone class of major tranquilizers. typiske neuroleptika, såsom haloperidol, er potente dopamin-receptorantagonister og anbefales kun at blive brugt som en læges sidste udvej til behandling af chorea.


Drug Class Summary
Depression er forholdsvis almindelige hos patienter med HD og bør behandles farmakologisk så hurtigt som diagnosen depression er givet. Depression hos forsøgspersoner med HD kan administreres de samme midler, der anvendes til behandling af depression af andre årsager. 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.

Paroxetine (Paxil)
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.