Objective: To report the findings of reduced striatal dopamine transporter (DAT) density in patients with Sydenham’s chorea (SC) in remission assessed by single-photon emission computed tomography (SPECT) with [99mTc]TRODAT-1.

Background: Sydenham’s chorea is characterised by an immune-mediated striatal dysfunction caused by cross-reactive antibodies to beta-hemolytic streptococcal antigens. The presence of subtle bradykinesia in patients with a history of SC in remission suggests the possibility of a long standing nigro-striatal dysfunction in the disease.

Methods: We performed striatal DAT density with SPECT imaging using [99mTc]TRODAT-1 in three patients with SC in remission. Chorea was quantified using the UFMG Sydenham’s Chorea Rating Scale and parkinsonism with UPDRS. Clinical data was recorded by reviewing charts.

Results: We report three adult patients with a history of childhood acute generalised chorea (onset at seven, thirteen and fourteen years old). All patients had generalised chorea and hypotonia. One also showed subtle bilateral bradykinesia when tapping the fingers. Hyperactivity and learning difficulties were present in one patient. All patients had normal rheumatologic tests and two patients showed mitral valve thickening on echocardiogram. All subjects were treated with valproic acid and had remission of chorea in up to three years after the disease onset. Recurrence of chorea occurred in one patient, and the patient was noted to have generalised chorea, worse on the right side, and mild bilateral bradykinesia. Chorea improved with reintroduction of valproic acid. Currently, all patients are asymptomatic and under no treatment for at least three years. Despite being in remission and without medication, grade one bradykinesia was noticeable when tapping fingers in all patients, either bilaterally or unilaterally. TRODAT-1 SPECT showed reduced DAT density bilaterally in all patients.

Conclusions: Our data suggests that the pathophysiology of SC can potentially include dopaminergic dysfunction as shown by the finding of decreased DAT density in the striatum of patients with SC in remission.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/evidence-of-striatal-dopaminergic-dysfunction-sydenhams-chorea-in-remission-with-99mtc-trodat-1-spect/