Epilepsy Eyelid Myoclonia Jeavons Syndrome
What is Epilepsy with Eyelid Myoclonia?
Epilepsy with Eyelid Myoclonia is a rare form of generalized epilepsy that can have several possible seizure types. It is also known as Jeavons syndrome.
- Eyelid myoclonia with or without absence seizures:
- Eyelid myoclonia is the most common seizure type. These consist of brief and repeated myoclonic jerks of the eyelids, eyeballs roll upwards, and the head may move slightly backwards.
- These events usually last less than 6 seconds but can happen many times per day.
- They are often induced or brought about by flashing lights or closing the eyes.
- If a person has a brief loss of awareness with the eyelid myoclonia, the seizure is called eyelid myoclonia with absence.
- About 1 out of 5 people can develop eyelid myoclonic status epilepticus. This happens when eyelid myoclonia and absences occur back-to-back over a prolonged period.
- Generalized tonic-clonic seizures:
- Generalized tonic-clonic seizures are seen infrequently and occur mostly in older children and adolescents. They are usually provoked by lack of sleep or light stimulation.
- Myoclonic jerks of the arms and legs may occur but are not frequent.
Seizures in children with Epilepsy with Eyelid Myoclonia (Jeavons syndrome) are frequent and occur multiple times a day.
Seizures in this syndrome are often hard to completely control.
- Medication works better for generalized tonic-clonic seizures than for eyelid myoclonia.
- If seizure medicines control a person's generalized tonic-clonic and absence seizures, other medication trials to improve isolated eyelid myoclonia often don't help or may not be needed.
This syndrome is typically life-long. It rarely goes away and people will need seizure medicines throughout their life.
While many people with Epilepsy with Eyelid Myoclonia are intellectually normal, some can have varying degrees of learning problems.
About 1 in 10 people have a history of febrile seizures.
How common is Epilepsy with Eyelid Myoclonia?
This syndrome is very rare and accounts for only 1-2% of people seen at specialty epilepsy centers.
What causes Epilepsy with Eyelid Myoclonia?
The cause of this epilepsy syndrome is unknown. A family history of seizures or epilepsy, usually generalized epilepsy, has been reported in up to 80% of children with this seizure type.
How is Epilepsy with Eyelid Myoclonias diagnosed?
- A good history is done by your health care provider. A detailed description of what happens during your seizures is very helpful. (Find forms to help you describe your seizures in our toolbox.)
- An EEG (electroencephalogram) is done.
- In many instances, a prolonged video EEG is also required.
- The video EEG is the most important test and will help in making the diagnosis of Epilepsy with Eyelid Myoclonia.
- During the EEG, you (or your child) will be asked to repeatedly close and open your eyes and photic stimulation with a strobe light will be used.
- Between seizures, brief bursts of fast (3-6 Hz) generalized polyspike-and-wave are typical on the EEG.
- Eye closure and photic stimulation activates the EEG and often elicits eyelid myoclonia with or without absences.
- The EEG abnormality is also brought about by hyperventilation.
- MRI (magnetic resonance imaging) scans are usually normal, but may be done to exclude a brain abnormality.
- Genetic testing is usually negative.
How is Epilepsy with Eyelid Myoclonia treated?
Seizures in Epilepsy with Eyelid Myoclonia tend to be resistant to anti-seizure medication.
- Medications often tried are levetiracetam (Keppra), valproic acid (Depakote/Depakene), ethosuximide (Zarontin), lamotrigine (Lamictal), topiramate (Topamax), and clobazam (Onfi), amongst others. Medications are typically used in combination.
- Medicines such as oxcarbazepine (Trileptal/Oxtellar XR), carbamazepine (Tegretol/Epitol), lacosamide (Vimpat), and phenytoin (Dilantin/Phenytek) are typically not used as they may worsen seizures.
- Children with this form of epilepsy will benefit from using commercially available Zeiss (Z1) blue lenses. In many instances, the episodes may reduce significantly when using these lenses.
- The ketogenic diet may also be tried.
What is the outlook for people with Epilepsy with Eyelid Myoclonia?
- Epilepsy with Eyelid Myoclonia is a lifelong disease.
- Generalized tonic-clonic and absence seizures respond better to treatment than eyelid myoclonia.
- International League Against Epilepsy (ILAE) on epilepsy with eyelid myoclonias
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