- Starts in mid childhood with the first seizure occurring between 3 and 10 years old.
- Boys and girls are equally affected.
- Typically children will become pale, complain of feeling sick and usually vomit during the seizure. Some children will have eye deviation and tonic-clonic movements during a seizure.
- More than half of the seizures will occur in sleep.
- Seizures tend to last longer than normal, from 20-60 minutes.
- It's not genetically determined.
- EEG is the most important test used to diagnosis this syndrome.
What is Panayiotopoulos Syndrome?
Panayiotopoulos Syndrome (PS), also known as early onset occipital epilepsy, is a common childhood epilepsy syndrome with partial (also called focal) seizures. It emerges in mid-childhood usually between the ages of 3-10 years. Both boys and girls can develop PS.
- Children with PS have normal physical and cognitive development.
- The seizure symptoms of PS are frequently mistaken as non-epileptic conditions such as encephalitis, syncope, migraine, cyclic vomiting syndrome, motion sickness, sleep disorder, or gastroenteritis.
What do the seizures in PS look like?
Seizures in PS usually start as partial or focal seizures that can spread to a generalized seizure. During a seizure, a child may look pale, complain of feeling sick and may vomit. Some children will have eye deviation (eyes turn to one side) and tonic-clonic movements may occur.
More than half of the seizures will occur in sleep particularly in the first hour after falling asleep. Seizures tend to be prolonged, sometimes lasting 20 to 60 minutes.
Is Panayiotopoulos Syndrome inherited?
PS is not genetically determined and does not usually run in families.
How is Panayiotopoulos Syndrome diagnosed?
The EEG (electroencephalogram) is the most important test in making a diagnosis of PS. MRI scans are usually normal in a child with PS.
- The EEG in children with PS who are not being treated with seizure medications shows abnormal spikes in one or both occipital lobes.
- Sometimes centro-temporal spikes are seen. This means that spikes are seen in the motor area and temporal lobe of the brain.
- A ‘fixation off sensitivity’ may be seen with PS. Epileptiform or seizure discharges are seen on an EEG when the child’s eyes are closed or if they are not focusing (or fixating) on an object. When the child opens their eyes or focuses (fixates) on an object, the seizure discharges on the EEG will go away.
- Sometimes a child may be thought to have PS but has a normal routine EEG. It’s critical that any child with a question of PS have an EEG done during sleep and when awake. This is the only way to make certain that the diagnosis has been accurately assessed.
An example of an EEG in a child with PS
How is Panayiotopoulos Syndrome treated?
Seizures in some children with PS may be infrequent. In these children, seizure medication may not be needed.
When seizures occur more frequently the seizures can be controlled with anti-seizure medications like oxcarbazepine (Trileptal), carbamazepine (Tegretol or Carbatrol), levetiracetam (Keppra), gabapentin (Neurontin), zonisamide (Zonegran), lacosamide (Vimpat), and others.
Children with PS who have long seizures may need emergency medical treatment or treatment with a rescue therapy, such as diazepam rectal gel (Diastat) or another form on benzodiazepine given into the nose (intranasally) or under the tongue.
- Parents of children with PS should talk to the treating neurologist or health care provider to learn about seizure emergencies.
- Talk to the health care team about what kind of rescue therapy could be used and when to use it.
- When seizures last longer than usual or if a generalized seizure lasts too long (generally considered 5 minutes or longer), a child may need emergency medical care.
What is the outlook for persons with Panayiotopoulos Syndrome?
Generally the outlook for children with this syndrome is good.
- Children with PS will have normal physical and cognitive development despite prolonged seizures.
- Nearly all children will stop having seizures 2-3 years after the first seizure.
- The risk of epilepsy in adult life is not higher than in the general population.