Intravenous Immunoglobulins as Effective Treatment in Sydenham's Chorea

INTRODUCTION Acute Rheumatic Fever (ARF), chronic rheumatic heart disease (RHD) and neuropsychiatric/ movement disorders related to group A beta haemolytic streptococcal infections (GABAS) remain major public health problems in South Africa. RHD may require repeated hospital admissions, invasive surgery and often results in premature death. In Africa ARF has a mortality of 2-3%. 1 Likewise, neuropsychiatric disorders such as Sydenham's Chorea (SC) resulting from GABAS result in school difficulties, frequent absenteeism, behaviour problems such as obsessive compulsive disorder and may progress to Tourette Syndrome (personal experience). Patients may suffer from disabling neurologic and psychiatric symptoms many years after their initial episode of ARF (personal experience). The morbidity from these conditions demands enormous resources from the state in terms of medical and surgical treatment and we believe results in preventable stress and suffering to the patient and his or her family. Whilst the ideal goal is primary prevention a measure of secondary prevention has been attained by the use of monthly prophylactic penilente. In cases where ARF has occurred, some further intervention is desirable, to prevent the above complications.

At the Rheumatic Fever Clinic (RFC) at Red Cross Hospital patients with established RHD and old cases of Sydenham's Chorea ( SC) are seen. There are 2-4 new cases of ARF seen per month. ARF remains a notifiable disease but it appears that these new cases are often not notified. (personal communication PAWC health officials) SC is a major manifestation of ARF and following the 1992 update of the modified Jones Criteria, it is sufficient alone to make a diagnosis of ARF.2 The duration of SC ranges from a few weeks to 2 years. Recurrences occur in one third of patients.3 Current treatment of SC is purely symptomatic and whether it resolves in 6 weeks or 2 years is dependent on innate healing mechanisms within the individual. SC is postulated to be mediated through auto- antibodies that mis-recognise neuronal cell components. These complexes then result in an inflammatory response in the basal ganglia with the resultant clinical picture of SC. 3 We hypothesise, that the use of intravenous immunoglobulins ( IVIG) will decrease the number of circulating antibodies thereby decreasing symptom severity and shortening the course of the illness. Prophylactic penicillin must be continued to prevent re-exposure and further formation of antibodies.

AIM We aim to determine whether treatment of SC with IVIG expedites recovery and thus cuts down on morbidity and frequent follow up.

METHOD Our aim is to collect a minimum of 20 patients with acute SC, diagnosed according to the revised Jones Criteria for ARF and with an absence of indicators of other causes of chorea. (Table 1) The study would run for 4 years or until the minimum number are recruited.

Patients would be identified and the chorea would be rated as mild, moderate or severe and all signs of chorea carefully documented as per attached format. (A) Special investigations would be performed (Table 2). Patients with IgA deficiency would be excluded from the study. Informed consent would be obtained. Patients would be randomly selected to receive IVIG and or standard treatment. Each patient will be allocated a code number.