Alternating Hemiplegia of Childhood

Alternating Hemiplegia of Childhood

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Alternating hemiplegia of childhood (AHC) is a neurological disorder that requires lifelong care. Because it is rare, with symptoms that mimic other conditions, an accurate diagnosis is essential. Duke is among the few hospitals with specialists dedicated to treating children with alternating hemiplegia of childhood. Our experts create detailed plans to address your child's concerns, manage their symptoms, and improve their quality of life. 

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About Alternating Hemiplegia of Childhood

ATP1A3 Gene Mutation
Around 75 percent of people with alternating hemiplegia of childhood have a mutation in a specific gene, ATP1A3. This gene mutation is usually spontaneous, meaning it rarely runs in families.

AHC Symptoms Begin Before 18 Months of Age
Symptoms include recurring episodes of paralysis that may attack one side of the body or both sides at once. Episodes may also include painful muscle stiffness and uncontrollable eye movements. Up to half of children with AHC eventually develop epilepsy.

Complications Can Vary
Complications often include learning disabilities, behavioral issues, sleep disorders, and physical problems such as trouble walking or swallowing. People with AHC also have a higher risk of developing heart problems later in life. 

AHC Affects Many Aspects of a Child's Development
Because AHC affects so many aspects of a child’s development, people with alternating hemiplegia of childhood benefit when they are diagnosed and managed by a team of specialists with a deep understanding of the disorder. 

Our Locations
Duke Health offers locations throughout the Triangle. Find one near you.

Tests and Procedures

The sooner we identify alternating hemiplegia of childhood, the sooner we can help manage your child’s symptoms and coordinate treatments that maximize their developmental potential.

Comprehensive Evaluation

We conduct a thorough medical history, which is key to diagnosing alternating hemiplegia of childhood.

Genetic Tests

Genetic testing can confirm the presence of the ATP1A3 gene mutation, which is associated with most cases of AHC. This testing can also rule out mutations associated with other neurological disorders. Advanced genetic analysis and counseling is often provided. You may also have the option to participate in ongoing genetic research.

Electroencephalogram (EEG)

EEG tests measure electrical activity in the brain. They help confirm or rule out epilepsy.


An echocardiogram looks for structural abnormalities of the heart.


An electrocardiogram may look for heart rhythm abnormalities.

Sleep Studies

Sleep studies can identify sleep abnormalities such as obstructive sleep apnea, central sleep apnea, restless legs, etc.

MRI and Other Imaging Tests

Tests like MRI can detect or rule out structural brain problems and other medical conditions that can cause episodes of paralysis or muscle stiffness.

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Although there is no cure for alternating hemiplegia of childhood, certain treatments help control symptoms and address physical or mental challenges.


Flunarizine can help reduce the frequency, severity, and duration of attacks of paralysis and muscle stiffness. While it is not FDA approved or marketed in the USA, we are able to offer  it through a special permissions process. Other medications given during an attack induce sleep, which often relieves symptoms. And children who also have epilepsy may need medication to manage seizures.

Vagus Nerve Stimulation

People with AHC are prone to epilepsy, and seizures may be difficult to control. An implanted device called a Vagus Nerve Stimulator (VNS) provides electrical stimulation along the vagus nerve, which can often be helpful in drug-resistant epilepsy.

Ketogenic Diet

Studies have shown that a diet of very low carbohydrates and high amounts of fat can reduce seizures in children with epilepsy.

Physical, Occupational, or Speech Therapy

Children with alternating hemiplegia of childhood usually need physical or occupational therapy to strengthen muscles, improve balance, increase mobility, or learn more efficient ways of performing everyday tasks. And speech therapists help children who have trouble speaking, eating, or swallowing.

Best Children's Hospital in NC
Duke Children's Hospital & Health Center is proud to be nationally ranked in nine pediatric specialties.

Why Choose Duke

A Comprehensive Approach 
Many specialists contribute to your child’s evaluation and care plan. All team members -- including specialists in neurology, cardiology, child behavioral health, medical genetics, neurodevelopment, neuropsychology, occupational therapy, physical therapy, psychiatry, sleep medicine, and speech therapy -- have extensive experience and specialize in caring for children with AHC.

Experience with AHC and Related Rare Conditions
Duke is uniquely positioned to diagnose and manage atypical alternating hemiplegia of childhood, meaning AHC that doesn't meet specific criteria or isn't caused by the classic ATP1A3 gene mutation. We also treat other rare conditions caused by different mutations in the same gene -- febrile induced paroxysmal weakness and encephalopathy (FIPWE), epileptic encephalopathy, relapsing encephalopathy with cerebellar ataxia (RECA), and CAPOS syndrome -- as well as other conditions associated with ATP1A3.

Influential Research
Duke doctors and researchers discovered the ATP1A3 gene mutation that is now known to be a leading cause of AHC. This 2012 breakthrough paved the way for additional research that may lead to effective new treatments and possibly a cure. 

Access to Clinical Trials
Your child may be eligible to participate in ongoing clinical trials as a patient at Duke. We are the lead center for the International AHC Research Consortium’s OBSERV-AHC study. This long-term effort will monitor the natural history of AHC, to better understand the factors that determine health outcomes. Our involvement in research helps us test promising new treatments, such as gene replacement therapy, before they’re approved for study or use in humans.

We Work with Your Doctor
Families travel to Duke from across the country and around the world for alternating hemiplegia of childhood care. We work closely with your local doctor, so your child receives the most comprehensive care possible no matter where you live.

Lifelong Care
Access to care at Duke doesn’t end when your child reaches adulthood. We continue to see patients of all ages who are affected by AHC. 

Support Services
We understand families may be traveling to Duke from far away, and alternating hemiplegia of childhood evaluations can take several days. We can arrange local lodging and coordinate transportation to and from the appointments. And through our partnership with Cure AHC,  it is possible to meet other parents and children affected by AHC while receiving care at Duke. 

Reviewed: 11/19/2018