Chorea in children: etiology, diagnostic approach and management
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HIGH IMPACT REVIEW IN NEUROSCIENCE, NEUROLOGY OR PSYCHIATRY - REVIEW ARTICLE
Chorea in children: etiology, diagnostic approach and management José Fidel Baizabal‑Carvallo1 · Francisco Cardoso2 Received: 30 April 2020 / Accepted: 1 August 2020 © Springer-Verlag GmbH Austria, part of Springer Nature 2020
Abstract Chorea is defined by the presence of abnormal, involuntary, continuous, random movements that results from a number of autoimmune, hereditary, vascular, metabolic, drug-induced and functional (psychogenic) causes. Chorea may present at all stages of life, from newborns to elderly individuals. While Huntington disease is the main suspicion in adults presenting with chorea, once a drug-induced or parkinsonian dyskinesia have been ruled out; Huntington disease exceptionally presents with chorea in children. Sydenham chorea is considered the most common cause of acute childhood-onset chorea, but its prevalence has decreased in Western countries. However, in younger children other etiologies such as dyskinetic cerebral palsy, anti-NMDAR receptor encephalitis, other autoimmune conditions, or mutations in NKX2-1, ADCY-5, FOXG1, GNAO1, GPR88, SLC2A1, SQSTM1, ATP8A2, or SYT-1 should be considered. In this manuscript, we review the main causes, diagnosis and management of chorea in children. Keywords Chorea · Children · Infancy · Autoimmune · Sydenham
Introduction Chorea is a movement disorder (MD) defined by continuous flow of unpredictable sequence of one or more discrete involuntary movements or movement fragments (Sanger et al. 2010; Cardoso et al. 2006). It is sometimes reported simultaneously with athetosis characterized by slow, smooth, sinuous, writhing movements, predominantly involving the hands, preventing maintenance of a stable posture (Lanska 2013). The term “athetosis” was introduced in 1871 by the American physician William Hammond (Lanska 2013). Although it has been dismissed as a form of post-hemiplegic chorea or a mixture of chorea and dystonia (Lanska 2010), it is still used frequently in modern literature. A number of etiologies can manifest with chorea, however, they vary according to the age at onset (Table 1). In this manuscript,
* José Fidel Baizabal‑Carvallo [email protected] 1
Department of Sciences and Engineering, University of Guanajuato, Ave León 428, Jardines del Moral, 37320 León, Guanajuato, Mexico
Movement Disorders Unit, Neurology Service, The Federal University of Minas Gerais, Belo Horizonte, MG, Brazil
2
we review the etiologies of chorea or choreoathetosis with onset at or before 15 years of age.
Methods We performed a systematic search in PubMed using the term “chorea” and combined with the terms “children”, “infant”, “cerebral palsy”, “autoimmune”, “hereditary”, “paroxysmal dyskinesia”, “metabolic”, “vascular”, “drug-induced”, “psychogenic” and “functional”. Papers in English and Spanish language were selected for this review. The final reference list was generated after selecting the manuscripts with key information regarding the selected topics.
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