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Published on: November 21, 2013
Chorea in children: etiology, diagnostic approach and management
José Fidel Baizabal-Carvallo1, Francisco Cardoso2
1Department of Sciences and Engineering, University of Guanajuato, Ave León 428, Jardines del Moral, 37320, León, Guanajuato, Mexico. baizabaljf@hotmail.com.
Insights
Chorea, characterized by involuntary movements, has diverse causes in children. This review covers the main etiologies, diagnostic approaches, and management strategies for pediatric chorea.
Area of Science:
- Neurology
- Pediatrics
- Genetics
Background:
- Chorea involves abnormal, involuntary movements with various causes including autoimmune, hereditary, and drug-induced factors.
- While Huntington disease is common in adults, it's rare in children, where Sydenham chorea was historically prevalent but is now decreasing.
- Pediatric chorea necessitates considering diverse etiologies beyond Sydenham chorea.
Purpose of the Study:
- To review the primary causes of chorea in children.
- To outline diagnostic methods for pediatric chorea.
- To discuss management strategies for chorea in pediatric populations.
Main Methods:
- Literature review of pediatric chorea etiologies.
- Analysis of diagnostic criteria for chorea in children.
- Synthesis of current management guidelines for pediatric chorea.
Main Results:
- Common causes of childhood chorea include dyskinetic cerebral palsy, anti-NMDAR receptor encephalitis, and autoimmune conditions.
- Genetic mutations in genes like NKX2-1, ADCY-5, and SLC2A1 are increasingly recognized causes.
- Accurate diagnosis relies on a comprehensive evaluation, including neurological examination, imaging, and genetic testing.
Conclusions:
- Pediatric chorea presents a diagnostic challenge due to its varied causes.
- Early and accurate diagnosis is crucial for effective management and improved outcomes.
- This review provides a framework for understanding and managing chorea in children.
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.
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