Sleep-Related Hypermotor Epilepsy (SHE)

Reviewed by: HU Medical Review Board | Last reviewed: August 2022

Sleep-related hypermotor epilepsy (SHE) is an epilepsy syndrome. Epilepsy is a disease of the brain (neurological disease). People with SHE have seizures mostly when they are asleep. SHE can have other names. It can also be called:1,2

  • Nocturnal frontal lobe epilepsy
  • Autosomal dominant frontal lobe epilepsy
  • Frontal lobe epilepsy

Who gets sleep-related hypermotor epilepsy?

People with SHE can begin having seizures any time from childhood to the age of 60. Most people are diagnosed before they are 20 years old.1

SHE is considered to be a rare condition. Many people with SHE inherited the condition. It can be caused by one or more faulty genes. It is estimated that about 30 percent of families are impacted by SHE caused by a faulty gene.1-3

Not much is known about the other possible causes of SHE. SHE may also be caused by other factors, such as a condition called focal cortical dysplasia (FCD). FCD happens when cells are abnormally organized in the brain.1-3

What are the symptoms of SHE?

The main symptom of SHE is seizures. Seizures in SHE normally happen during sleep. People with SHE can experience different types of seizures and symptoms in SHE, such as:1-3

  • Many seizures in one night
  • Seizures that last up to a few minutes but are mostly 30 seconds
  • Seizures that look like:
    • Waking up at night, perhaps confused
    • Having nightmares
    • Having night terrors
    • Movements such as twisting, walking, and thrusting
    • Shouting, moaning, or crying
    • Difficulty breathing or catching a breath
    • Staying aware but moving their arms and legs uncontrollably

How is sleep-related hypermotor epilepsy diagnosed?

It can be difficult to diagnose SHE, as it has similar symptoms to other epilepsy syndromes and movement disorders. If you have symptoms of SHE, your doctor will give you a physical exam and ask you about your full medical history, including family history. The doctor may also conduct tests to help with a diagnosis. These tests include:1

The results of some of these tests may come back normal. But even normal results help doctors used to exclude other similar conditions.1

How is SHE treated?

Anti-seizure drugs

People with SHE can take anti-seizure drugs to control their seizures. Most people respond well to these drugs. Common anti-seizure drugs used to treat SHE are:1-3

  • Oxcarbazepine (Trileptal®)
  • Carbamazepine (Tegretol® or Carbatrol®)
  • Levetiracetam (Keppra®)
  • Gabapentin (Neurontin®)

About 30 percent of people with SHE are not able to control their seizures with anti-seizure drugs. These people have drug-resistant SHE. An epilepsy specialist can help with other treatment options if anti-seizure drugs are not working.1,2


Surgery is an option for people with drug-resistant SHE. In epilepsy surgery, a part of the brain where seizures occur is removed. This is effective in epilepsy syndromes that occur in one part of the brain, like the frontal lobe.2,4

Surgery is not the first treatment option. But it can help when anti-seizure drugs have not worked. Some studies have shown that people with drug-resistant SHE can have their seizures fully controlled (eliminated) after surgery. For other people, surgery can reduce the number of seizures they have.2,4

Living with SHE

SHE is a lifelong condition but does not get worse over time. Most people with SHE have normal brain function other than seizures. Some people with SHE can have mood disorders or issues with their behavior. Some people can also have problems with their mental abilities (intellectual disability), such as a harder time:1,5

  • Thinking
  • Learning
  • Problem-solving

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