Treatment of Sydenham Chorea with Plasma Exchange or Prednisone

This study is currently recruiting patients.

Sponsored by

National Institute of Mental Health (NIMH)

Purpose

Sydenham chorea (SC) is an uncommon autoimmune neuropsychiatric disorder. It is a result of an abnormal immune reaction to a bacterial throat infection (streptococcal pharyngitis). Children with this disorder have abnormal bodily movements (chorea) and psychiatric symptoms, particularly mood swings (emotional lability), inattention, and obsessive compulsive symptoms. Although thought to be a mild self resolving disorder, children with SC can be severely disabled for many months as a result of the movements, may have multiple recurrences over the subsequent years, and can suffer from disabling cardiac, neurologic, and psychiatric symptoms for many years after the initial episode. Presently there is no cure for Sydenham chorea. Some medical treatment exists for control of the movement symptoms. Previous research comparing the steroid prednisone (PDN), intravenous immunoglobulin (IVIG), and plasma exchange (PEX), has shown IVIG and PEX to be superior choices for treatment of the first episode of SC. However, the results are preliminary findings and have not yet reached statistical significance. Researchers will stop testing steroid therapy and lower the amount of patients involved in the study in order to achieve statistical significance. This study will continue to compare the remaining 2 therapies for SC, IVIG and PEX.

Study Type: Clinical Trial

Official Title: Treatment of Sydenham Chorea with Plasma Exchange or Prednisone

Further Study Details: Sydenham chorea (SC) is an uncommon autoimmune neuropsychiatric disorder. It arises from an abnormal immune response to a streptococcal pharyngitis. Children with this disorder present with both abnormal movements (chorea) and psychiatric symptoms, particularly emotional lability, inattention and obsessive compulsive symptoms. Although thought to be a mild and self-resolving disorder, children with SC can be severely disabled for many months as a result of the movements, may have multiple recurrences over the subsequent years and can suffer from disabling cardiac, neurologic and psychiatric symptoms many years after their initial episode. Little is known about the underlying neural substrate of this disorder. Recent work with transcranial magnetic stimulation (TMS) suggests that cortical inhibitory systems may be abnormal in patients with chorea. However, it appears that the nature of these abnormalities differs depending on the TMS parameter being tested. Thus, post-excitatory inhibition (also referred to as the cortical silent period [CSP]) is increased in patients with Huntington Disease (HD), while intracortical inhibition is decreased. Patients with obsessive compulsive disorder also show decreased intracortical inhibition, but there is little change in the CSP. In preliminary studies, we have shown that, in contrast to situation with HD, the CSP is shortened in patients with SC. There is no information regarding intracortical inhibition in these latter patients. Since patients with SC have both chorea and obsessive compulsive symptoms, it is reasonable to suggest that this intracortical system will also be abnormal. Insights into the cortical physiology of SC will lead to a greater understanding of chorea and may lead to improved treatments for this and other choreatic disorders. At the present time, treatments for this disorder are restricted to symptomatic control of the movements. The most effective and most frequently prescribed of these medications are the neuroleptics such as haloperidol and pimozide. Although immune modulation with corticosteroids has been attempted for SC, there have been no controlled trials. Evidence arising from these published case series suggests that the resolution of the symptoms may have arisen as part of the natural history of the disorder rather than as a response to the steroids. With the advent of newer and perhaps, more effective immunomodulatory treatments we have carried out a randomized, open-label trial comparing prednisone (PDN), intravenous immunoglobulin (IVIG), and plasma exchange (PEX) as acute treatment for the initial episode of SC. Preliminary results have shown a clinically meaningful superiority of PEX and IVIG compared to PDN, but this has not reached statistical significance. We intend to continue the treatment trial using only two arms: PEX and PDN. In addition to decreasing the numbers needed to achieve statistical significance, this change is also a practical response to the national shortage of IVIG.  

Eligibility

Genders Eligible for Study:  Both Criteria

No neurologic or psychiatric illnesses and must be free of ADHD symptoms.

No albinism or a personal or family history of sensorineural hearing loss.

Location and Contact Information

Maryland

National Institute of Mental Health (NIMH), 9000 Rockville Pike   Bethesda,   Maryland,    20892,   United States; Recruiting

PRPL     Warren G. Magnuson Clinical Center National Institutes of Health   Bethesda,    Maryland,   20892-4754,   United States  1-800-411-1222    prpl@mail.cc.nih.gov  

More Information

Detailed Web Page

Publications that lead up to this study

Allen. 1995. Case study: A new infection-triggered, autoimmune subtype of pediatric OCD and Tourette's syndrome, J Am Acad Child Adolesc Psychiatry, Vol. 34, p. 307

Swedo. 1994. Sydenham's chorea: A model for childhood autoimmune neuropsychiatric disorders, JAMA, Vol. 272, p. 1788

Zabriskie. 1985. Rheumatic fever: the interplay between host, genetics and microbe, Circulation, Vol. 71, p. 1077

Study ID Numbers  92-M-0132

NLM Identifier  NCT00001321

Date study started March 25, 1992

Recruitment status verified  March 20, 2000

Last Updated  March 20, 2000

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