Overview
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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 lớn treating children with alternating hemiplegia of childhood. Our experts create detailed plans lớn address your child's concerns, manage their symptoms, and improve their quality of life.
Find an Alternating Hemiplegia of Childhood Doctor
About Alternating Hemiplegia of Childhood
ATP1A3 Gene Mutation
Around 75% of people with alternating hemiplegia of childhood have a mutation in a specific gen, ATP1A3. This gen mutation is usually spontaneous, meaning it rarely runs in families.
AHC Symptoms
Symptoms begin before 18 months of age and include recurring episodes of paralysis that may attack one side of the body toàn thân or both sides at once. Episodes may also include painful muscle stiffness and uncontrollable eye movements. Symptoms can last for minutes lớn days and are relieved with sleep. Up lớn half of children with AHC eventually develop epilepsy.
Complications
Complications often include developmental delays, learning disabilities, gastrointestinal issues including constipation, behavioral issues, epilepsy, and sleep disorders. Other problems include trouble walking, swallowing disorders, and involuntary movements. People with AHC also have a higher risk of developing heart problems later in life.
AHC Affects Development
Because AHC affects so sánh 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 in Durham.
Tests and Procedures
Treatments Overview
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
Description
We conduct a thorough medical history, which is key lớn diagnosing alternating hemiplegia of childhood.
Genetic Tests
Description
Genetic testing can confirm the presence of the ATP1A3 gen 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 available. You may also have the option lớn participate in ongoing genetic research.
Electroencephalogram (EEG)
Description
EEG tests measure electrical activity in the brain. They help confirm or rule out epilepsy.
Echocardiogram
Description
An echocardiogram looks for structural abnormalities of the heart.
Electrocardiogram
Sleep Studies
Description
Sleep studies can identify sleep abnormalities such as obstructive sleep apnea, central sleep apnea, restless legs, and others.
MRI and Other Imaging Tests
Description
Tests lượt thích MRI scans 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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Treatments
Treatments Overview
Although there is no cure for alternating hemiplegia of childhood, certain treatments help control symptoms and address physical or mental challenges.
Medication
Description
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 U.S., we are able lớn 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 lớn manage seizures.
Vagus Nerve Stimulation
Description
People with AHC are prone lớn epilepsy, and seizures may be difficult lớn 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
Description
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
Description
Children with alternating hemiplegia of childhood usually need physical or occupational therapy lớn strengthen muscles, improve balance, increase mobility, or learn more efficient ways of performing everyday tasks. 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 lớn be nationally ranked in 10 pediatric specialties.
Why Choose Duke
A Comprehensive Approach
Duke has a team of specialists who contribute lớn 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 lớn diagnose and manage atypical alternating hemiplegia of childhood -- AHC that doesn't meet specific criteria or isn't caused by the classic ATP1A3 gen mutation. We also treat other rare conditions caused by different mutations in the same gen, including 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 gen mutation that is now known lớn be a leading cause of AHC. This 2012 breakthrough paved the way for additional research that may lead lớn effective new treatments and possibly a cure.
Access lớn Clinical Trials
Your child may be eligible lớn 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 gen replacement therapy, before they’re approved for study or use in humans.
We Work with Your Doctor
Families travel lớn Duke from across the country and around the world for alternating hemiplegia of childhood care. We work closely with your local doctor, so sánh your child receives the most comprehensive care possible no matter where you live.
Lifelong Care
Access lớn care at Duke doesn’t kết thúc when your child reaches adulthood. We continue lớn see people of all ages who are affected by AHC.
Support Services
We understand families may be traveling lớn Duke from far away, and alternating hemiplegia of childhood evaluations can take several days. We can help you arrange local lodging and coordinate transportation lớn and from the appointments. And through our partnership with Cure AHC, it is possible lớn meet other parents and children affected by AHC while receiving care at Duke.
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