P3C314: Beyond the Dance: Exploring the Neuropsychiatric Manifestations of Sydenham's Chorea

Introduction: A 16 year-old presented to the ED with acute onset of insomnia, pressured speech, involuntary extremity movements, pharyngitis, and dysphagia for one week. The patient was found to have positive Group A streptococcus (GAS) PCR, elevated inflammatory markers, and presentation consistent with Sydenham’s chorea, and diagnosed with acute rheumatic fever. They also had mania-spectrum symptoms which are not typically described in Sydenham’s chorea.

Case Description: A 16 year-old with ADHD, anxiety, and asthma presented to the ED with acute onset of insomnia, pressured speech, involuntary extremity movements, pharyngitis and dysphagia for one week.The patient and mother reported school field trip the month prior where multiple individuals had pharyngitis as well as right hip surgery due to chronic hip pain three weeks prior to presentation. They initially presented to their pediatrician over a week prior for worsening hyperactivity for weeks, thought to be related to their ADHD so their stimulant was increased. For a week prior to ED presentation, they reported elevated mood, decreased need for sleep (2-3 hrs of sleep/night), distractibility, talkativeness with pressured speech, tangential racing thoughts, irritability, disinhibition with inability to filter speech, and word-finding difficulties. Initial ED vitals were notable for BP 135/78, HR 151. Exam revealed non-interruptible pressured speech, involuntary choreiform movements, right hand pill-rolling movements, and tongue-thrusting. Initial work-up revealed WBC 18.2, CRP 205, ESR 68. CSF studies demonstrated normal cell count, protein, and glucose. GAS PCR was positive. Brain MRI/MRA and echocardiogram were normal.

Pediatric psychiatry consult-liason team recommended holding home stimulant and SSRI given concern for mania-spectrum symptoms secondary to Sydenham’s. The patient met Jones criteria for acute rheumatic fever with one major criteria (chorea) and two minor criteria (elevated inflammatory markers, polyarthralgia). Cephalexin was recommended for GAS treatment due to penicillin allergy. After initiating antibiotics and sleeping for the first time in a week, exam was notable for improved pressured speech and involuntary movements, with ongoing difficulty sustaining grip with constant squeezing of playdoh, difficulty writing their name, and difficulty drawing "10 past 2" on a clock (Figure 1).

While hospitalized, they passed an oral challenge to penicillin for prolonged prophylaxis. They were discharged with significant improvement in physical and neuropsychiatric symptoms of acute rheumatic fever.

Discussion: While other neuropsychiatric manifestations of Sydenham’s chorea such as OCD, anxiety, depression, psychosis, and ADHD, have been reported, mania-spectrum symptoms such as elevated mood, insomnia, irritability, have not yet been documented in pediatric populations.

Conclusion: To date, this is one of the first reports of mania-spectrum symptoms as a neuropsychiatric manifestation of Syndenham’s chorea in a child. Early recognition of Sydenham's chorea as a potential organic cause of neuropsychiatric symptoms, including mania-spectrum symptoms, can lead to improved clinical course.