Controlling Seizures in Angelman Syndrome

Emily Malcolm, PhD avatar

by Emily Malcolm, PhD |

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Angelman syndrome is a rare genetic disorder characterized by neurological and developmental problems, among other symptoms. More than 90% of patients experience epileptic seizures, which can be dangerous.

However, several methods can help control seizures in patients with Angelman syndrome.

Medications

Many common anti-seizure medications can effectively treat seizures associated with Angelman syndrome. These include levetiracetam, lamotrigine, and clobazam. Valproate, clonazepamphenobarbital, and topiramate may also be used but may have more side effects.

Some medications are ineffective and may even exacerbate seizures. These medications, which should not be used for Angelman syndrome patients, are carbamazepine, oxcarbazepine, and vigabatrin.

Ketogenic diet

ketogenic diet — high in fat and low in carbohydrates — is sometimes effective at controlling or reducing the number of seizures, especially in children who do not respond to medication. Before starting any diet, talk to both a registered dietitian and your treatment team to establish the best diet for your child.

Vagus nerve stimulation

Vagus nerve stimulation (VNS) is a procedure where a small device applies an electrical stimulus to the vagus nerve to try and halt seizures before they happen. VNS may be effective for patients with Angelman syndrome, especially in addition to anti-seizure medications. The surgery involves implanting a device similar in size to a pacemaker. The device sends regular electrical signals to the vagus nerve in the neck to disrupt seizure activity in the brain.

Gene therapy

Although still experimental, researchers think that gene therapy for Angelman syndrome may be able to reduce seizure activity. In this approach, the idea is to introduce a healthy copy of the mutant gene that causes Angelman syndrome into patients’ cells. Supplying the healthy UBE3A gene is thought to potentially reduce or even abolish the symptoms, including seizures.

 

Last updated: June 25, 2020

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