Sydenham's chorea in a girl with juvenile idiopathic arthritis treated with anti‐TNFα therapy

Cimaz, Rolando; Gana, Simone; Braccesi, Giulia; Guerrini, Renzo

doi:10.1002/mds.22923

Cited by 7 publications

(3 citation statements)

References 55 publications

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“…It is also notable that when narcolepsy with cataplexy is misdiagnosed, a choreiform movement disorder is often proposed as the cause: PANDAS and Sydenham's chorea were previous diagnoses in our case series and an initial diagnosis of Huntington's chorea was reported in the first paediatric case series (Yoss and Daly, 1960). Moreover, typical characteristics of narcolepsy with cataplexy are reported in Sydenham's chorea, such as being ‘clumsy’, ‘withdrawn’, ‘troubled’, ‘day-dreaming’, ‘transient intellectual impairment’ and ‘having nightmares’ (Gatti and Rosenheim, 1969; Cimaz et al ., 2010). Strikingly, motor impersistence demonstrated by ptosis or tongue protrusion is a common sign in Sydenham's chorea, a condition also frequently associated with grimacing and hypotonia (Oosterveer et al ., 2010), and recurs in ‘cataplectic facies’ (Serra et al ., 2008; Dhondt et al ., 2009; Merino-Andreu and Martinez-Bermejo, 2009).…”

Section: Discussionmentioning

confidence: 99%

Complex movement disorders at disease onset in childhood narcolepsy with cataplexy

Plazzi

Pizza

Palaia

et al. 2011

Brain

166

137

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Narcolepsy with cataplexy is characterized by daytime sleepiness, cataplexy (sudden loss of bilateral muscle tone triggered by emotions), sleep paralysis, hypnagogic hallucinations and disturbed nocturnal sleep. Narcolepsy with cataplexy is most often associated with human leucocyte antigen-DQB1*0602 and is caused by the loss of hypocretin-producing neurons in the hypothalamus of likely autoimmune aetiology. Noting that children with narcolepsy often display complex abnormal motor behaviours close to disease onset that do not meet the classical definition of cataplexy, we systematically analysed motor features in 39 children with narcolepsy with cataplexy in comparison with 25 age- and sex-matched healthy controls. We found that patients with narcolepsy with cataplexy displayed a complex array of ‘negative’ (hypotonia) and ‘active’ (ranging from perioral movements to dyskinetic–dystonic movements or stereotypies) motor disturbances. ‘Active’ and ‘negative’ motor scores correlated positively with the presence of hypotonic features at neurological examination and negatively with disease duration, whereas ‘negative’ motor scores also correlated negatively with age at disease onset. These observations suggest that paediatric narcolepsy with cataplexy often co-occurs with a complex movement disorder at disease onset, a phenomenon that may vanish later in the course of the disease. Further studies are warranted to assess clinical course and whether the associated movement disorder is also caused by hypocretin deficiency or by additional neurochemical abnormalities.

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Section: Discussionmentioning

confidence: 99%

Complex movement disorders at disease onset in childhood narcolepsy with cataplexy

Plazzi

Pizza

Palaia

et al. 2011

Brain

166

137

View full text Add to dashboard Cite

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“…We identified 1479 patients with IPD (median [IQR] age at onset, 10 [8-13] years in 1354 patients; 985 of 1426 [69.1%] female and 441 of 1426 [30.9%] male) were identified from 307 articles…”

Section: Resultsmentioning

confidence: 99%

Treatments and Outcomes Among Patients with Sydenham Chorea

Eyre,

Thomas,

Ferrarin

et al. 2024

JAMA Netw Open

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ImportanceSydenham chorea is the most common acquired chorea of childhood worldwide; however, treatment is limited by a lack of high-quality evidence.ObjectivesTo evaluate historical changes in the clinical characteristics of Sydenham chorea and identify clinical and treatment factors at disease onset associated with chorea duration, relapsing disease course, and functional outcome.Data SourcesThe systematic search for this meta-analysis was conducted in PubMed, Embase, CINAHL, Cochrane Library, and LILACS databases and registers of clinical trials from inception to November 1, 2022 (search terms: [Sydenham OR Sydenham’s OR rheumatic OR minor] AND chorea).Study SelectionPublished articles that included patients with a final diagnosis of Sydenham chorea (in selected languages).Data Extraction and SynthesisThis study followed the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline. Individual patient data on clinical characteristics, treatments, chorea duration, relapse, and final outcome were extracted. Data from patients in the modern era (1945 through 2022) were entered into multivariable models and stratified by corticosteroid duration for survival analysis of chorea duration.Main Outcomes and MeasuresThe planned study outcomes were chorea duration at onset, monophasic course (absence of relapse after ≥24 months), and functional outcome (poor: modified Rankin Scale score 2-6 or persisting chorea, psychiatric, or behavioral symptoms at final follow-up after ≥6 months; good: modified Rankin Scale score 0-1 and no chorea, psychiatric, or behavioral symptoms at final follow-up).ResultsIn total, 1479 patients were included (from 307 articles), 1325 since 1945 (median [IQR] age at onset, 10 [8-13] years; 875 of 1272 female [68.8%]). Immunotherapy was associated with shorter chorea duration (hazard ratio for chorea resolution, 1.51 [95% CI, 1.05-2.19]; P = .03). The median chorea duration in patients receiving 1 or more months of corticosteroids was 1.2 months (95% CI, 1.2-2.0) vs 2.8 months (95% CI, 2.0-3.0) for patients receiving none (P = .004). Treatment factors associated with monophasic disease course were antibiotics (odds ratio [OR] for relapse, 0.28 [95% CI, 0.09-0.85]; P = .02), corticosteroids (OR, 0.32 [95% CI, 0.15-0.67]; P = .003), and sodium valproate (OR, 0.33 [95% CI, 0.15-0.71]; P = .004). Patients receiving at least 1 month of corticosteroids had significantly lower odds of relapsing course (OR, 0.10 [95% CI, 0.04-0.25]; P &lt; .001). No treatment factor was associated with good functional outcome.Conclusions and RelevanceIn this meta-analysis of treatments and outcomes in patients with Sydenham chorea, immunotherapy, in particular corticosteroid treatment, was associated with faster resolution of chorea. Antibiotics, corticosteroids and sodium valproate were associated with a monophasic disease course. This synthesis of retrospective data should support the development of evidence-based treatment guidelines for patients with Sydenham chorea.

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“…In fact, a multi-national survey of botulinum toxin injectors and patients who were receiving onabotulinumtoxinA or abobotulinumtoxinA for CD indicated that 55% of physicians and 70% of patients prefer shorter injection intervals than those intervals actually received. 56,57 A retrospective chart review study in 102 patients with CD who had been under continuous care for 1 year and 10 months (January 1, 1998, to August 31, 1999) found that the mean duration of efficacy, inferred to be the time between repeat injections, was a mean (range) of 15.5 (12.2-24.3) weeks. 58 Long-term patterns of onabotulinumto-xinA treatment for CD were also evaluated in a retrospective survey study in 133 patients over 6 years (also discussed above).…”

Section: Studies Of Variable Bont-a Administration Patternsmentioning

confidence: 99%

Botulinum Toxin Therapy for Cervical Dystonia: The Science of Dosing

Evidente¹,

Pappert

2014

Tremor and Other Hyperkinetic Movements

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The first-line treatment for cervical dystonia (CD) is botulinum toxin type A (BoNT-A), which has been established as a highly effective and well-tolerated therapy. However, this treatment is also complex and challenging to apply in clinical practice. Approximately 20% of patients discontinue therapy due to treatment failure, adverse effects, and other reasons. In addition, expert consensus recommendations are lacking to guide physicians in the optimal use of BoNT-A for CD. Among the issues still to be clarified is the optimal dosing frequency. The generally accepted standard for intervals between BoNT-A injections is >12 weeks; however, this standard is based primarily on the methodology of pivotal trials for the BoNT-A products, rather than on evidence that it is optimal in comparison to other intervals. While some retrospective, observational studies of BoNT-A used in clinical practice appear to support the use of >12-week dosing intervals, it is often unclear in these studies how the need for reinjection was determined. In contrast, a prospective dose-ranging trial in which patients were allowed to request reinjection as early as 8 weeks showed that about half of patients receiving abobotulinumtoxinA, at the currently recommended initial dose of 500 U, requested reinjection at 8 weeks. Moreover, results from an open-label, 68-week extension phase of the pivotal trial of incobotulinumtoxinA showed that 47.1% of patients had received reinjection at #12 weeks. Ongoing studies, such as the Cervical Dystonia Patient Registry for Observation of BOTOXH Efficacy (CD PROBE), may help clarify this question of optimal dosing intervals for BoNT-A in CD.

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Sydenham's chorea in a girl with juvenile idiopathic arthritis treated with anti‐TNFα therapy

Cited by 7 publications

References 55 publications

Complex movement disorders at disease onset in childhood narcolepsy with cataplexy

Complex movement disorders at disease onset in childhood narcolepsy with cataplexy

Treatments and Outcomes Among Patients with Sydenham Chorea

Botulinum Toxin Therapy for Cervical Dystonia: The Science of Dosing

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