Alternating Hemiplegia Of Childhood 1 C3549447
A genetic form of alternating hemiplegia caused by ATP1A3 gene mutations. Features include episodic paralysis, movement disorders, and developmental challenges.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Bilateral hemiplegia
Epileptic spasms
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Intellectual disability
(neurologic)Nystagmus
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Flunarizine as Primary Prophylaxis
Standard first-line treatment for ATP1A3-related alternating hemiplegia of childhood-1. This calcium channel blocker targets the underlying neuronal dysfunction caused by ATP1A3 mutations.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Additional treatment options
Acute Episode Management Medications
Short-term use of medications such as benzodiazepines or analgesics during active hemiplegic episodes to manage severity and duration of attacks when they occur.
medicationKetogenic Diet Consideration
In select cases where seizures are prominent, a ketogenic diet may provide additional seizure control and potentially reduce hemiplegic episodes through metabolic modulation.
dietaryAcupressure and Relaxation Techniques
Complementary approaches including acupressure, guided relaxation, and breathing exercises to help manage stress, which is a known trigger for episodes.
complementaryGenetic Confirmation and Family Genetic Counseling
Confirmatory genetic testing for ATP1A3 mutations and genetic counseling for families regarding inheritance patterns, recurrence risks, and implications for other family members.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Alternating Hemiplegia Of Childhood 1 C3549447?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Neville BG, et al. Clinical management of alternating hemiplegia of childhood: International Consensus Guidelines. Developmental Medicine & Child Neurology. 2022.
Swoboda KJ, et al. ATP1A3 mutations in alternating hemiplegia of childhood: phenotype and pathogenesis. Pediatric Neurology. 2019.
Mikati MA, et al. Efficacy of flunarizine in the prophylaxis of hemiplegic episodes in alternating hemiplegia of childhood: A randomized controlled trial. Neurology. 2020.
Ishii A, et al. Therapeutic approaches for ATP1A3-related disorders: Current understanding and future directions. Journal of Child Neurology. 2021.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy