Table 1 List of compounds used to treat hyperkinesia in Huntington’s disease

Tetrabenazine

Starting dose: 2 × 12,5 mg

Target dose: up to 3 × 75 mg per day

max. daily dose ~ 200 mg

If sedative side effects occur, consider dividing the medication into four separate doses, with the primary dosage administered as the nighttime medication

Depression/suicidality, sedation, sleep disorders, and extrapyramidal side effects, Parkinsonism, akathisia

Rare cases of a neuroleptic malignant syndrome

Do not combine with MAO inhibitors!

Tiaprid

Starting dose: 2 × 50 mg

Target dose: up to 4 × 300 mg pro Tag

Max. Daily dose ~ 300 to 1000 mg. One study used up to 3000 mg daily

If sedative side effects occur, consider dividing the medication into four separate doses, with the primary dosage administered at nighttime

Side effects similar to another classic

dopamine D2 receptor antagonists, in particular sedation and Parkinsonism

Atypical neuroleptics, e.g. risperidone, olanzapine and aripiprazole

Similar to psychiatric indications

Only smaller studies and case reports

Occasionally Parkinsonism

monitoring for impulsivity and

impulse control disorders for aripiprazole

(Risperidone may also be helpful for the treatment of irritability; Olanzapine may also be beneficial for the treatment of weight loss)

Haloperidol

Similar to psychiatric indications 5 to 10 mg/day (as evening dose) sufficient in most cases

Side effects of a classic

Dopamine receptor antagonists, esp.

Parkinsonism

not drug of the first choice

possibly useful in cases with psychosis or aggressive behavior

Amantadine

100 to 400 mg daily dose (divided into 2 to 4 single doses)

Data partially contradictory Caution: Psychosis! (consensus strength 80%)

Valproate

effect dose-dependent

Rarely indicated in myoclonic Hyperkinesia (action myoclonus; [6, 10, 53])

Levetiracetam

Up to 2 × 1500 mg per day

Only small studies and case reports Parkinsonism, sedation, cognitive disorders, increased irritability!