Abstract
Introduction: Alternating hemiplegia of childhood (AHC) is a rare neurological disorder characterized by a wide and heterogeneous range of clinical manifestations that begin before the age of 18 months. The objective of this review was to explore the available literature on AHC, with an emphasis on its clinical, etiopathogenic, and therapeutic aspects, through an unstructured qualitative narrative review that would allow for a broad and comprehensive understanding of the topic, beyond the classical inclusion criteria.
Materials and methods: A scoping review of the literature was conducted to search for epidemiology, etiopathogenesis, clinical manifestations, diagnosis, and treatment approaches for AHC. A literature search was performed on PubMed, SciELO, LILACS and BIREME with no restrictions on language or publication date.
Results: The initial search identified 371 studies, of which those addressing only adults were excluded. Abstracts and full texts were reviewed, resulting in the inclusion of 29 articles.
Discussion: The prevalence of AHC is 1 in 1,000,000 in children and is associated with variants in ATP1A3, which is responsible for the Na+/K+ ATPase pump and regulation of neuronal excitability. Clinically, it presents as transient paroxysmal episodes of paresis or hemiplegia, preceded by environmental stress, bathing, or psychological factors, and may be associated with cognitive function deterioration, autonomic dysfunction, altered consciousness, epilepsy, dystonia, ataxia, and choreoathetosis. The diagnosis is primarily based on clinical criterio, and the presence of a genetic variant confirms the diagnosis. Treatment involves managing paroxysmal events and comorbidities, including medications such as benzodiazepines, flunarizine, and topiramate.
Conclusion: AHC is a rare but complex disease that requires a multidisciplinary approach. Multicenter, controlled, and standardized studies with long-term follow-up are needed to better understand the nature of this disease.
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