Jeavons Syndrome

Reviewed by: HU Medical Review Board | Last updated: July 2022

Jeavons syndrome or epilepsy with eyelid myoclonia is a rare type of generalized epilepsy. Generalized epilepsy affects the entire brain. Several types of seizures can occur in Jeavons syndrome.1

Jeavons syndrome usually starts in children between ages 2 and 14. But it is typically lifelong. Girls are more likely than boys to have Jeavons syndrome.1

What types of seizures occur in Jeavons syndrome?

Different types of seizures can happen in Jeavons syndrome. They are frequent events and can occur many times in the day.1

Eyelid myoclonia seizures, with or without absence

Eyelid myoclonia is a type of seizure that causes quick, uncontrolled jerking and twitching of the muscles around the eyes. Your head might move backward slightly. The twitching muscles can cause your:1,2

  • Eyelids to close quickly
  • Eyelids to flutter
  • Eyeballs to roll upward

The eyelid twitching usually lasts less than 6 seconds but can happen many times in the day. Flashing lights or closing your eyes can bring on these episodes. If you briefly lose awareness, the seizure is called myoclonia with absence.1

Myoclonic status epilepticus is a condition where you have back-to-back eyelid myoclonia and absence over an extended period of time. This happens in about 1 out of 5 people with these types of seizures.1

Generalized tonic-clonic seizures

The old term for these seizures is "grand mal." These seizures can be frightening to have and to witness. They are the type most people think of when someone says they have seizures.2-4

Tonic-clonic seizures have phases:3,4

  • Tonic phase – Stiffening, loss of consciousness, falling to the floor
  • Clonic phase – Violent jerking
  • Postictal phase – Feeling confused and disoriented

Tonic-clonic seizures are not common in Jeavons syndrome. When they do occur, it is mostly in older children and adolescents. They can be brought on by exposure to bright lights or not getting enough sleep.1

What causes Jeavons syndrome?

Doctors do not know the exact causes of Jeavons syndrome. But genes may play a role. In up to 8 out of 10 children with this seizure type, there is a family history of seizures or epilepsy. The most common family history is generalized epilepsy.1

Symptoms

The seizures of Jeavons syndrome are brief and frequent. Symptoms will vary but include:1,5

  • Continuous blinking
  • Eyeballs rolling upward toward the back of your head
  • Backward head movement
  • Decreased awareness of surroundings

Diagnosis

First, your doctor will take your medical history. This is the core of diagnosis. Your doctor needs to have all the details about before, during, and after your seizures started. Think of it as putting together a puzzle – every piece needs to be there to see the whole thing.1,6

You might not remember all the details or answers to your doctor's questions. Bring a friend or family member who has seen the seizures and can help piece your symptoms together.1,6

EEG

Your doctor will order an EEG, or electroencephalogram. An EEG is a painless test that records your brain's electrical activity. You will most likely need a video EEG for the doctor to diagnose Jeavons syndrome. With a video EEG, you will be asked to open and close your eyes while a strobe light flashes.1,7,8

If you have Jeavons syndrome, you will have an abnormal EEG recording when closing your eyes and when the light is flashing.1

Other tests

You will likely have magnetic resonance imaging (MRI) of your brain. If you have Jeavons syndrome, the MRI will help your doctor rule out other conditions. You may also have genetic tests.1

Treatment for Jeavons syndrome

Seizures from Jeavons syndrome are usually not well controlled with anti-seizure drugs. But your doctor will prescribe different anti-seizure drugs to see if any single type or combination works for you.1

Some children with Jeavons syndrome may have fewer episodes when using Zeiss Z1 blue lenses. Talk to your child’s doctor about where to find these.1

A diet that is very low in carbohydrates and high in fat (ketogenic or "keto" diet) might help. This diet helps some but not all people with seizures. Talk to your doctor or nutritionist before starting a new diet.1

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