Treatment of Sydenham’s Chorea: A Review of the Current Evidence

Shannon L. Dean; Harvey S. Singer

doi:10.5334/tohm.376

Figures & Tables

Table 1

Symptomatic Treatments

Drug and Dose	Number of patients	Age of Patients (years)	Chorea Duration Before treatment	Study Design	Assessment Tool	Outcome	Adverse Reactions	Study
HLP vs. PMZ, dosage unknown	29 followed (unclear distribution)	6–17	2 d–11 y	Retrospective comparison	Qualitative	Response HLP 14.5 d PMZ 29.5 d Remission HLP 43 d PMZ 110 d	HLP: dystonia, Parkinsonism, sleepiness, forgetfulness PMZ: headache sleepiness, dry mouth, numbness	Demiroren et al.20
HLP 2–4 mg/d	2	5–12	3–6 w	Case report	Qualitative	Response in 48 h	None	Axley39
HLP 1–3 mg/d	4	8–15	2 w–8 y	Case series	Qualitative	Response in 3–4 d	None	Shenker et al.33
PMZ 2 mg bid	1	14	2 y	Case report	Qualitative	Response in 2 d	None	Harries-Jones and Gibson37
PMZ 2 mg bid	2	12–16	2 w–6 m	Case report	Qualitative	Response “immediate” Remisson 2 w	None	Shannon and Femichel35
CLP? dose	91	Unclear	Unclear	Retrospective comparison	Unclear	unclear	Parkinsonism	Teixiera et al.61
HLP: 0.5–15 mg/d	HLP 82, HLP+ PR 5, CLP 1, unknown 12	2–36	4 d–8 y	Retrospective case series	Qualitative	82/87 response HLP 5/5 response HLP +PR	Not reported specifically	Tumas et al.31
OLZ 5–10 mg/d	6	5–13	8–10 w	Case series	Qualitative	Remission 2–3 w	None	Sethi et al.46
VPA 250 mg bid	1	19	2 months	Case report	Qualitative	Response 1 d Remission 1 m	None	Mclauchlan22
VPA 15–25 mg/kg/d	5	11–18	5 d–2.5 y	Case series	Qualitative	Remission 5–10 d	None	Dhaneraj et al.21
VPA 20 mg/kg/d	1	8	3 w	Case report	Qualitative	Response 12 h	None	Alvarez and Novac19
VPA 15–25 mg/kg/d	15	5–13	1–104 w	Case series	Qualitative	Response 4–8 d in 13/15 pts	Severe hypotonia	Daoud et al.36
VPA 20–25 mg/kg/d	9	11	Unknown	Case series	Qualitative	Response 11 d	None	Davutoglu et al.30
VPA 20 mg/kg/d	10	9	2 w–1 y	Case series	Blinded observer	Response 3–7 d Remission 1–2 w	None	Sabui and Pant23
VPA 25 mg/kg/d	1	15	3 m	Case report	Qualitative	No response after 3 w	None	Appleton and Jan34
VPA 600–800 mg/d	2	12–12	2–4 d	Case report	Qualitative	Response 4–7 d Remission 12 d	None	Steinberg et al.45
CBZ 4–10 mg/kg/d	10	7–16	7 d–8 m	Prospective case series	Qualitative)	Response, 2–14 d Remission 2–4 w	Pruritic rash	Harel et al.24
CBZ 15–20 mg/kg/d	2	8–8	10 d–3 m	Case report	Qualitative	Response 1 w Remission 1–2 m	None	Roig et al.44
CBZ 15–20 mg/kg/d, HLP 3 mg/d, VPA 20 mg/kg/d	HLP 6, VPA 6, CBZ 6	7–15	4 d–8 y	Comparison study	Qualitative	Response 6/6 VPA, 5/6 CBZ, 3/6 HLP	HLP: somnolence, dystonic reaction	Peña et al.25
CBZ 15 mg/kg/d, VPA 20–25 mg/kg/d	CBZ 17 VPA, 7	5–14	2 d–6 m	Comparison study	Qualitative	Response CBZ 4–14 d, VPA 2–30 d Remission CBZ 3–20 w VPA 1–25 w	None	Genel et al.26

[i] Abbreviations: bid, Twice a Day; CBZ, Carbamazepine; CLP, Chlorpromazine; d, Days; HLP, Haloperidol; m, Months; OLZ, Olanzapine; PMZ, Pimozide; PR, Prednisone; pts, Patients; VPA, Valproic Acid; w, Weeks.

Table 2

Immunosuppressive Treatments

Drug and Dose	Number of patients	Age of Patients (years)	Chorea Duration Before treatment	Study Design	Assessment Tool	Outcome	Adverse Reactions	Study
PR ×2 mg/kg/day ×3 w with taper	5	4–11	2–30 d	Case series	Qualitative	Response 24–48 h remission 7–12 d	None	Barash et al.32
CR IV 30–40 u/d ×3–9 d, taper PR 30–75 mg/d for 5–10 d, taper	8	6–10	2 w-7 m	Retrospective case series	Qualitative	Response in 3–5 d	None	Green27
MP IV 25 mg/kg/d ×5 d DF 0.9 mg/kg/d ×3 m	10	7–11	15 d	Prospective case series	USCRS	Response 48 h, Remission 21 d	None	Fusco et al.53
MP IV 25 mg/kg/d (children) or 1 g/d (adults) ×5 d, PR taper	5	11–46	Not specified	Case series	Unique 4-point scale	Response 5 d	Cushing’s syndrome	Cardoso et al.56
MP IV 25 mg/kg/day ×5 d, 1 mg/kg PR taper	5	4–12	4–8 w	Case series	USCRS	Chorea subscale decreased from mean of 14 to mean 8 at 1 month	Weight gain, moon face	Teixiera et al.56
PR 2 mg/kg/d ×4 w 25 day taper	22 PR, 15 placebo	7–11	2–84 d	Prospective double blind randomized control trial	Chorea intensity scale	Significant difference in weekly score	Weight gain, moon face	Paz et al.49
IVIG 400 mg/kg ×5 d	2	11–13	2 -2 m	Case report	Qualitative	Remission “several” days	None	van Immerzeel et al.28
2 g/kg IVIG	1	10	14 d	Case report	Qualitative	Response 4 d	None	Boresma et al.52
2 g/kg IVIG	1	13	1 w	Case report	USCRS	UCSRS 45 onset, 18 3 weeks, 2 at 3 months	None	Mohammad et al.54
HLP 0.025–0.05 mg/kg±1 g/kg IVIG ×2 days	10 HLP, 10 IVIG+HLP	5–13	7–140 d	Open label randomized comparison study	Unique 16 point scale	HLP +IVIG improved scores at 1,3 and 6 months	None	Walker et al.67
PLEX × 5 d	1	16	Not specified	Case report	Unspecified	Response 1 m	None	Miranda et al.29
IVIG 1 g/kg/d ×2 d, PLEX ×5 days PR 1 mg/kg ×10 d	4 IVIG, 8 PLEX 6 PR	5–14	Not specified	Open label randomized comparison	Mean Chorea Severity Scale	Response in all (no clear statistical difference)	PR: weight gain, moon face	Garvey et al.51

[i] Abbreviations: CR, Corticotropin; d, Days; DF, Deflacort; h, Hours; IVIG, Intravenous Immunoglobulin; m, Month; MP, Methylprednisolone; PLEX, Plasmapheresis; PR, Prednisone; USCRS, Universidade Federal de Minas Gerais Sydenham’s Chorea Rating Scale; w, Weeks.

Treatment Recommendations. Our suggested approach to treatment is presented.

Treatment of Sydenham’s Chorea: A Review of the Current Evidence

Figures & Tables

Table 1

Symptomatic Treatments

Table 2

Immunosuppressive Treatments

Figure 1

Treatment Recommendations. Our suggested approach to treatment is presented.

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