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Published on: October 14, 2021
[Acute Chorea]
Patricia Herrera-Mora1, Vania S Kenai Cruz-Rio1, Raúl Gonzalez-Gonzalez2
1Departamento de Neurología, Instituto Nacional de Pediatría, Ciudad de México, México.
Abstract:
Chorea is a hyperkinetic movement disorder characterized by involuntary, irregular, unpredictable, and purposeless movements that may involve the face, trunk, and limbs, with variable intensity and speed. In childhood, acute-onset chorea represents a neurological emergency, as it may reflect potentially treatable underlying conditions. From a pathophysiological perspective, chorea is associated with an imbalance between the direct and indirect pathways of the basal ganglia, particularly involving the striatum, resulting in excessive facilitation of motor activity. Pediatric chorea can be broadly classified into hereditary and acquired forms, with acquired causes being the most frequent. Postinfectious and autoimmune etiologies, especially Sydenham chorea, predominate, followed by infectious, metabolic, vascular, toxic, drug-induced, and paraneoplastic causes. Diagnostic evaluation relies on accurate identification of the movement phenomenology, assessment of temporal profile, and a comprehensive clinical examination, supported by targeted laboratory tests, neuroimaging, and genetic studies when appropriate. Treatment is primarily directed toward the underlying etiology and symptomatic control of involuntary movements, most commonly through pharmacologic modulation of dopaminergic pathways, combined with a multidisciplinary approach. Prognosis varies according to etiology and is generally favorable in the most common pediatric forms; however, recurrences and persistent neuropsychiatric symptoms may occur, highlighting the need for longterm follow-up.
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