Overview
Alternating hemiplegia of childhood (AHC) is a rare, severe neurological disorder characterized by recurrent episodes of unilateral or alternating hemiplegia, dystonia, and other movement disorders 1. These episodes can be triggered by various factors and often lead to developmental delay and intellectual disability 1.Diagnosis
Diagnosis is primarily clinical, based on the characteristic pattern of recurrent hemiplegic episodes 1.
Genetic testing for mutations in the ATP1A3* gene is highly recommended for definitive diagnosis 1.
Neuroimaging (MRI) may be used to rule out other causes of hemiplegia but is typically normal in AHC 1.Management
No cure exists for AHC, and management focuses on symptomatic treatment and seizure control 1.
First-line treatment:
* Flunarizine is often used to reduce the frequency and severity of attacks 1.
* Topiramate is another option for seizure control and potentially reducing attack frequency 1.
Adjunctive treatments:
* Benzodiazepines may be used acutely to stop prolonged attacks 1.
* Other antiepileptic drugs may be used for seizure management 1.
* Physical and occupational therapy are important for managing motor deficits 1.Special Populations
Pediatrics: AHC is a childhood-onset disorder 1.Key Recommendations
Consider flunarizine for reducing the frequency and severity of hemiplegic attacks 1. (Evidence: Moderate)
Genetic testing for ATP1A3 mutations should be performed for definitive diagnosis 1. (Evidence: Moderate)
Utilize symptomatic and supportive care, including physical and occupational therapy, to manage motor deficits 1. (Evidence: Moderate)References
1 Fernandes AER, Roveda JRC, Fernandes CR, Silva DF, de Oliveira Guimarães IC, Lima EM et al.. Relationship between nocturnal enuresis and sleep in children and adolescents. Pediatric nephrology (Berlin, Germany) 2023. link