TABLE 1.
Study characteristics of included studies in this review
| Study, Year | Study design | Patients characteristics | Drugs with dosage | Distribution of drugs | Outcomes | Side Effects |
|---|---|---|---|---|---|---|
| Direk et al. (2020)* | Retrospective |
N = 140 F = 98 M = 42 Age (years)= 11.8 ± 2.8 (6.0–16.7) Hemichorea= 45 Chorea= 95 Duration of chorea until admission (days)= 28.2 ± 25.8 |
Dopamine antagonist=HLP Antiepileptic=VPA, LEV, and CBZ |
HLP= 29 CBZ= 36 Na‐VPA= 60 LEV= 15 |
Response Time= HLP:1‐6M, CBZ: 2‐4W, VPA: 1‐2W Response Time as 2nd choice= VPA: 1‐2W, CBZ 2‐4W, LEV 1‐6M Remission Time= Similar between all drugs |
Mild Drowsiness= Na‐VPA: 2, CBZ: 4, HLP: 4 Increased Appetite= Na‐VPA:5 Nausea= CBZ: 3 Dizziness= CBZ: 4, HLP: 4 |
| Araujo et al. (2002)* | Observational |
N = 20 Age (years)= 8 (6.0–12.0) F= 13 M= 7 |
Dopamine antagonist= HLP Antiepileptic= VPA Immunomodulators= Prednisone Others= DZP and SLP |
HLP= 12 VPA= 2 PR= 1 DZP= 5 SLP= 0 |
Recurrence=Symptomatic Treatment: 4, PR: 0 | Sedation occurred in 1/3rd people treated with symptomatic drugs |
| Demiroren et al. (2007) | Retrospective | N= 29 | Dopamine antagonist= HLP and PMZ | HLP vs. PMZ |
Response Time=HLP:14.5 ± 10.7d, PMZ: 29.5 ± 42.9d Remission Time=HLP 42.7 ± 29.9d, PMZ: 109.5 ± 115.5d Total Time of drug use=HLP: 51 ± 22.5d, PMZ: 84.3 ± 102.6d |
HLP= 3 (dystonia, Parkinsonism, sleepiness, absentmindedness, and forgetfulness PMZ= 1 (sweating, sleepiness, headache, dry mouth, and numbness) |
| Favaretto et al. (2020) | Retrospective Observational |
N= 30 Ageb (years)= 7.5 Prednisone Ageb (years)= 7.0 F= 13 M= 2 Standard therapy (PMZ or VPA) Ageb (years)= 9.0 F= 10 M= 5 |
Immunomodulators= PR (2 mg/kg/day) Antiepileptic= PMZ Dopamine antagonist= VPA |
PR= 15 Standard care= 15 |
Response Time= PR: 4d, Standard therapy: 16d Remission Time= PR: 30d, Standard therapy: 125d Relapse= PR: 1, Standard therapy: 3 |
Not reported |
| Gebremariam (1999; 2006) | Comparison study |
N= 18 Age (years)= 9.2 ± 2.63 (5–13.25) M= 10 F= 8 |
BPG vs. control |
Response= No significant difference Recurrence= BPG 0, Control 10 |
||
| (1999) & Genel et al. (2002) | Prospective Comparison |
N = 24 Age (years)= 11.3 ± 2.3 (5–14) Duration of complaints (days)= 24.6 ± 37.3 (2–180) F = 15 M = 9 Sodium Valproate Age (years)=12.4 ± 1.5 F= 5 M= 2 Carbamazepine Age (years)=10.9 ± 2.4 F=10 M=7 |
Antiepileptic= Na‐VPA (20 mg/kg/day), CBZ (15 mg/kg/day) |
CBZ= 17 Na‐VPA= 7 |
Response Time= CBZ: 7.4 ± 8.2d, Na‐VPA: 8.0 ± 4.0d Remission Time= CBZ: 10.1 ± 8.5w, Na‐VPA: 6.7 ± 6.3w Recurrence= CBZ: 3, Na‐VPA: 1 |
None |
| ( Paz et al. (2006) | RCT |
N= 37 M:F= 1.3:1 Hemichorea= 14 Chorea= 23 Prednisone M:F=1.2:1 Age (years)= 9.3 ± 1.9 Placebo M:F= 1.5:1 Age (years)= 10.5 ± 2.1 |
Dopamine antagonist= HLP |
PR=22 PLB=15 HLP=4 in PR, 7 in PLB |
Response Time=PR: 1 week Remission Time=PR:54.3±23.81, PB: 119.9±84.21 Recurrence=PR: 4, PB: 3 |
Weight gain Cushingoid appearance |
| Kulkarni and Anees (1996) | Prospective study |
N = 60 Age (years)= 11.1 (7–18) F= 36 M= 24 |
Antiepileptic= Na‐VPA (20 mg/kg/day), PBB (3 mg/kg/day) Dopamine Antagonist= HLP (0.05 mg/kg/day), CPZ (2 mg/kg/day) Others= DZP (0.2 mg/kg/day) |
Na‐VPA= 8 PBB= 13 HLP= 17 CPZ= 9 DZP=3 PBB and CPZ |
Response= PBB 13.7d, CPZ 17.9d, CPZ + PBB 21.8d, DZP 15d, HLP 12.6d, VPA 9.7d Recurrence= 13 (not specified) |
None |
| Garvey et al. (2005) & | RCT |
N= 18 F= 11 M= 7 Age= 10.2 ± 2.3 |
Immunomodulators= IVIG (1 g/kg), PE, PR |
IVIG= 4 PE= 8 PR= 6 |
Recurrence= IVIG: 3, PE: 2 |
IVIG= nausea:2, vomiting: 2, headache: 2, Hepatitis C:1 PE=Vasovagal episode without syncope: 2, citrate‐induced circumoral paresthesias: 1, Gram‐negative sepsis with Enterobacter cloacae: 1 PR= weight gain |
| Orsini et al. 2022) | Retrospective |
N= 171 F= 108 M= 63 Age= 9b |
Immunomodulators= IVIG, CS Antiepileptic= VPA Dopamine antagonist= HLP |
BZP= 151 CS= 59 IVIG= 3 CS + DA= 25 CS + AE= 22 AE= 18 DA= 8 AE + DA= 2 |
Remission Time= (unspecified) 6 months= 82 8 months= 80 Recurrence= 16 (unspecified) |
N/A |
| Peña et al. (2002) | Comparison study |
N= 32 F=10 M=8 Age= 11 ± 0.861 (11–15) |
Immunomodulators= IVIG, CS Antiepileptic= VPA (20 mg/kg/day), CBZ (15 mg/kg/day) Dopamine antagonist= HLP (3 mg/day) |
HLP= 6 VPA= 6 CBZ= 6 |
Response Time= HLP: 3 patients, 5 days Remission Time (Complete recovery)= VPA: 5 days, CBZ: 7 days Recurrence= CBZ: 3m, HLp: 10m |
HLP= excessive somnolence: 1, dystonic reaction= 1, failed to improve: 1 |
F: Female, M: Male, N: Number, AE: Anti‐epileptics, DA: Dopamine Antagonists, HLP: Haloperidol, VPA: Valproic Acid, LEV: Levetiracetam, CBZ: Carbamazepine, PR: Prednisolone, DZP: Diazepam, SLP: Sulpiride, PMZ: Pimozide, BPG: Benzathine Penicillin G, PBB: Phenobarbital, IVIG: Intravenous Immunoglobulin, PE: Plasmapheresis, CS: Corticosteroids.