Pharmacotherapy for Sydenham’s chorea: where are we and where do we need to be?

Overview of management of infection-related movement disorders with focus on specific-infections

“…Molecular mimicry is an immunological phenomenon where epitopes present on pathogens may mimic or can be similar to a peptide sequence of host antigens thereby misdirecting the activated host immunity to aberrantly act against the host antigen and resulting in autoimmune process. Sydenham chorea is a classic example of this phenomenon where it is shown that antibodies against group-A streptococcal carbohydrate cross-react with human neuronal cells resulting in chorea [8] , [45] . Second mechanism is epitope spreading where the initial highly specific immune response against an antigen gradually broaden during the inflammation to include new epitope in the same antigen or the release and presentation of “hidden” auto- antigens encountered during tissue injury.…”

“…Recurrences are not uncommon and can occur in 15–40 % of the cases with common triggers being poor adherence to secondary prophylaxis, oral contraceptive agents and pregnancy [60] . Management of SC involve three steps [8] : Antibiotic treatment for streptococcus Symptomatic control of the chorea Immunotherapy to control immune and inflammatory response …”

“…In patients where the chorea is mild and non-disabling, symptomatic therapy may not be required as these medications do not change the overall recovery or the recurrence rate of chorea [8] , [58] . When symptomatic therapy is considered, antiseizure medications (ASM) and dopamine antagonists are the two groups of medications commonly used.…”

“…Overall, both typical and atypical antipsychotics have good efficacy, but considering both efficacy and side effect profile, ASMs are preferred over dopamine antagonists [58] . Tetrabenazine (25 mg 2–3 times a day), has been tried in few cases with response noted over 2 weeks in 2 cases but no response in another [8] , [58] .…”

“…or specific infections such as movement disorders in human immunodeficiency virus infection, tuberculosis, neurocysticercosis, post infectious cerebellar ataxia etc. [5] , [6] , [7] , [8] , [9] . However, literature on IRMD prevalence, pattern, prognosis and management as a whole is lacking [1] , [4] , [10] , [11] .…”