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Hyperkinetic Movement Disorders
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Morales-Briceno Hugo, Victor S.C. Fung, Annu Aggarwal, Philip Thompson
HD is the most common cause of inherited chorea. It is autosomal dominant, with expanded CAG triplet repeats in the huntingtin (HTT) gene. A larger number of repeats correlates with earlier onset and severe disease. The usual onset for HD is in the fourth decade.
Post-viral syndromes
Published in Avindra Nath, Joseph R. Berger, Clinical Neurovirology, 2020
Anusha K. Yeshokumar, Eliza Gordon-Lipkin, Brenda Banwell
Sydenham’s chorea is an acute onset movement disorder characterized by non rhythmic, random movements following group A streptococcal pharyngeal infection and is a known manifestation of acute rheumatic fever. The history of Sydenham’s chorea dates back to the Middle Ages, when history texts described a “dancing curse” thought then to be placed by saints. Thomas Sydenham was the first to accurately describe this movement phenomenon in the late seventeenth century and in the nineteenth century. He described its association with rheumatic disease [56]. In the twenty-first century, while this clinical syndrome has become rare in developed countries, it remains an important cause of chorea not attributable to genetic etiology [57].
Anti-Phospholipid Antibodies: Clinical Complications Reported in Medical Literature
Published in E. Nigel Harris, Thomas Exner, Graham R. V. Hughes, Ronald A. Asherson, Phospholipid-Binding Antibodies, 2020
Bruyn and Padberg6 summarized neuropathological findings in their 11 patients. In only 3 patients were lesions of the basal ganglia evident and these were accompanied by thrombotic occlusion of vessel locally. More distant, cortical occlusions were evident in the other 8 patients. The paucity of pathological examinations on patients with chorea leads one only to speculate as to its pathogenesis without being able to draw any definite conclusions. The pathogenesis of CNS lupus, in any event, is poorly understood.
Pharmacotherapy for Sydenham’s chorea: where are we and where do we need to be?
Published in Expert Opinion on Pharmacotherapy, 2023
Roberta Bovenzi, Matteo Conti, Tommaso Schirinzi
If chorea is impairing, symptomatic therapy can be initiated. There is no unanimity on which is the best symptomatic treatment in SC. Most authors favor using CBZ (15 mg/kg/day) or VPA (20 mg/kg/day). TBZ may be a valid option, as younger patients are reported to have greater tolerance. The use of dopamine D2 receptor-blocking agents should be avoided, given their risk of extrapyramidal side effects. In mild-to-moderate cases, or when symptoms are persistent, most authors advise early introduction of oral corticosteroids such as prednisone or deflazacort. In moderate-to-severe cases of SC, pulse IV methylprednisolone therapy for 3–5 days should be the first-line treatment. IVIG and PE should be considered in refractory and severely disabling cases when all the other treatments have failed. In pregnant patients, CBZ and low doses of prednisone may be used to control most disabling cases. In addition to pharmacotherapies, patients with SC should be monitored closely for the onset of carditis [30]. Finally, patients with SC might also benefit from non-pharmacological measures, such as bed rest and supportive (social) care [21]. Figure 1. shows a practical approach to SC treatment, modified from Dean et al. [21].
Deutetrabenazine for tardive dyskinesia and chorea associated with Huntington’s disease: a review of clinical trial data
Published in Expert Opinion on Pharmacotherapy, 2019
Daniel O. Claassen, Michael Philbin, Benjamin Carroll
First-HD (NCT01795859) was a randomized, double-blind, placebo-controlled, parallel-group Phase 3 trial conducted by the Huntington Study Group (HSG) [4]. The study included 90 ambulatory adults diagnosed with HD who had a screening and baseline score ≥8 for total maximal chorea (TMC) on the Unified Huntington’s Disease Rating Scale (UHDRS) and a screening score ≥5 for UHDRS total functional capacity. The TMC score quantified chorea on a scale of 0–28 based on assessments of the face, bucco-oral-lingual area, trunk, and the four extremities. Higher scores were associated with more-severe cases of chorea. Patients with untreated or undertreated psychiatric illness were excluded. Patients were randomized 1:1 to receive deutetrabenazine or placebo. The duration of First-HD was 12 weeks, which included an 8-week phase during which patients were titrated to an optimal dose level, followed by a 4-week maintenance phase, after which there was a 1-week washout period. During the 8-week titration period, the dose could be increased in 6 mg increments on a weekly basis until adequate control of chorea was achieved or the maximum dose of 48 mg was reached. If a patient experienced a moderate-to-severe or serious AE related to the treatment, the dose could be reduced or suspended based on the investigator’s judgment. At the end of the titration period, the patient’s dose was established for the maintenance period.
Sydenham’s chorea: an update on pathophysiology, clinical features and management
Published in Expert Opinion on Orphan Drugs, 2019
Luiz Paulo Bastos Vasconcelos, Marcelle Cristina Vasconcelos, Maria Do Carmo Pereira Nunes, Antonio Lucio Teixeira
SC frequently presents as a monophasic and self-limiting condition, however persistence and recurrence of symptoms may occur. For instance, Cardoso et al. (1999) found that 50% of SC patients had chorea lasting more than 2 years, with female gender and carditis as factors associated with the persistence of SC [34]. On the other hand, Walker et al. (2007) reported a much lower duration of chorea in SC patients, even in untreated subjects. They followed cases of SC from a large cohort of RF patients and observed a mean chorea duration of 4.8 weeks in SC patients treated with prednisone and 11.7 weeks in untreated individuals [35]. Data inconsistencies regarding persistence of chorea might be due to regional differences in genetic susceptibility, selection bias, among other factors. The frequency of persistent SC is expected to be higher in studies performed in referral movement disorders clinics [34] compared to studies carried out in the community or in general pediatric clinics [35].