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Continuum (Minneap Minn) ; 25(4): 1001-1035, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31356291

RESUMO

PURPOSE OF REVIEW: This article provides an overview of the approach to chorea in clinical practice, beginning with a discussion of the phenomenologic features of chorea and how to differentiate it from other movement disorders. The diagnostic approach, clinical features of important acquired and genetic choreas, and therapeutic principles are also discussed. Practical clinical points and caveats are included. RECENT FINDINGS: C9orf72 disease is the most common Huntington disease phenocopy, according to studies in the European population. Anti-IgLON5 disease can present with chorea. The role of immunotherapies in Sydenham chorea has increased, and further clinical studies may be useful. Benign hereditary chorea is a syndrome or phenotype due to mutations in several genes, including NKX2-1, ADCY5, GNAO1, and PDE10A. New-generation presynaptic dopamine-depleting agents provide more options for symptomatic treatment of chorea with fewer adverse effects. Deep brain stimulation has been performed in several choreic disorders, but features other than chorea and the neurodegenerative nature should be taken into consideration. Studies on genetic interventions for Huntington disease are ongoing. SUMMARY: Clinical features remain crucial in guiding the differential diagnosis and appropriate investigations in chorea. Given the complexity of most choreic disorders, treating only the chorea is not sufficient. A comprehensive and multidisciplinary approach is required.


Assuntos
Coreia/diagnóstico por imagem , Coreia/genética , Adulto , Idoso , Idoso de 80 Anos ou mais , Antidiscinéticos/administração & dosagem , Proteína C9orf72/administração & dosagem , Proteína C9orf72/genética , Criança , Coreia/tratamento farmacológico , Diagnóstico Diferencial , Dopaminérgicos/administração & dosagem , Feminino , Humanos , Doença de Huntington/diagnóstico por imagem , Doença de Huntington/tratamento farmacológico , Doença de Huntington/genética , Masculino , Transtornos dos Movimentos/diagnóstico por imagem , Transtornos dos Movimentos/tratamento farmacológico , Transtornos dos Movimentos/genética , Neuroacantocitose/diagnóstico por imagem , Neuroacantocitose/tratamento farmacológico , Neuroacantocitose/genética , Fator Nuclear 1 de Tireoide/genética
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