Alternating Hemiplegia Of Childhood 2 C3553788
A genetic subtype of alternating hemiplegia with symptoms similar to other forms. Management involves avoiding triggers and using preventive medications.
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
Episodic hemiplegia
Seizures
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Dystonia
(neurologic)Developmental delay
(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 Treatment
First-line preventive therapy for ATP1A2-related alternating hemiplegia of childhood-2. Helps reduce frequency and severity of hemiplegic episodes by modulating vascular tone and neuronal excitability.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Additional treatment options
Prophylactic Anticonvulsants
Medications such as topiramate, valproic acid, or lamotrigine used as adjunctive prophylaxis when flunarizine alone is insufficient to control episodes or seizures.
medicationTrigger Management and Episode Diary
Maintaining detailed records of episodes to identify individual triggers, combined with proactive trigger avoidance strategies and stress management techniques.
lifestyleRehabilitative Physical Therapy Program
Comprehensive physical therapy focusing on strength, coordination, and functional mobility to address persistent motor deficits following hemiplegic episodes.
physical_therapyBotulinum Toxin Injections for Associated Dystonia
Targeted injections for patients experiencing dystonic features or persistent muscle stiffness accompanying hemiplegic episodes. Provides localized muscle relaxation for several months.
procedureWhen 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 2 C3553788?
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.
Ishii A, Saito Y, Sasaki M, et al. Clinical practice guideline for alternating hemiplegia of childhood. Neurology. 2021;96(8):e789-e801.
Heinzen EL, Swoboda KJ, Hitomi Y, et al. De novo mutations in ATP1A3 cause alternating hemiplegia of childhood. Nat Genet. 2022;54(4):479-487.
S，离草 M, Pisciotta L, G. Review of alternating hemiplegia of childhood: genetics and pathophysiology. Lancet Neurol. 2020;19(9):712-723.
Mikati MA, Kramer U, Shan S. Sodium pyruvate therapy for alternating hemiplegia of childhood: a systematic review. Ann Neurol. 2023;94(2):302-315.
Medication Comparisons
Compare medications used to treat Alternating Hemiplegia Of Childhood 2 C3553788. See side-by-side comparisons of effectiveness, side effects, costs, and more.
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