Intravenous immunoglobulin in acute Sydenham's chorea: A systematic review

Abstract

Sydenham's chorea (SC) is a major manifestation seen in 25% of patients with acute rheumatic fever. SC is the prototypic autoimmune neurological disorder, which has a less appreciated associated risk of psychiatric morbidity. We undertook a systematic review to examine whether the use of intravenous immunoglobulin affects clinical recovery and morbidity.

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

“…For example, reports varied on whether they provided the time to improvement of symptoms (10 out of 44),19,25,27,30,35–38,42,55 the time to full remission of symptoms (two out of 44),21,28 or both (13 out of 44) 20,22–24,26,32,33,35,41,44–46,52,53. Nine out of 38 studies reviewed improvement in a chorea score at fixed weekly, monthly, or yearly intervals, rather than reporting the time to an observed improvement or recovery 29,40,47–51,54,56. Twenty-eight out of 44 reported the total length of time treatment was provided 20,22–30,32–36,38,44–46,48–56…”

“…However, this literature review identified significant diversity of antibiotic treatment and/or reporting. Twenty-one studies failed to comment on whether primary or secondary penicillin treatment was utilized19–21,23,25,26,29,31,33–36,39,45,46,48,51,54–56 and 10 reports did not comment on the length of treatment or method of delivery 22,24,28,35,40,42–44,51,53. One study reported primary but not secondary penicillin treatment 47.…”

“…Case reports utilizing IVIG include a total of four patients who improved following the receipt of IVIG (a total of 2 gm/kg) over 5 days 28,52,54. One subject had failed valproic acid and haloperidol prior to treatment with IVIG, whereas the others were treated with IVIG as a primary therapy.…”

Systematic review of immunoglobulin use in paediatric neurological and neurodevelopmental disorders

“…Immunomodulation may benefit some children with paediatric autoimmune neuropsychiatric disorder associated with streptococcal infection, although the authors of the RCT studying this screened over 200 children before selecting their cohort, so the benefits of immunotherapy cannot be generalized to all patients with obsessive–compulsive disorder and tics . IVIG treatment in Sydenham chorea has previously been reviewed, with suggestions of future trials that could standardize time to treatment, control for additional treatments, assess psychiatric outcomes, have improved blinding at treatment and follow‐up, standardize dosing in line with other neuroinflammatory conditions, and have longer‐term follow‐up.…”

Uncontrollable movements of right upper and lower extremities in a child: A diagnostic puzzle

“…In moderate to severe cases, corticosteroids and intravenous immunoglobulins 15 . 16 have been shown to decrease the duration of illness through immune suppression. There is no established protocol for the symptomatic treatment for chorea, which is largely determined by the extent of disruption of daily activities.…”