What is Panayiotopoulos syndrome?
This syndrome is also known as early onset childhood occipital epilepsy.
Panayiotopoulos syndrome (PS) starts in early childhood, usually between the ages of 3-6 years, but children from 1-13 years have been described. Both boys and girls can develop PS. It occurs in approximately 3 out of 50 (6%) children between the ages of 1-15 who have epilepsy.
Children with PS mostly have normal physical and cognitive development.
Abnormalities that cause seizures and can be seen on EEG (electrocephalograph) commonly come from the occipital lobes, which is located in the back of the brain.
What do the seizures in PS look like?
- During a seizure, a child may look pale, complain of feeling sick and may vomit.
- They may faint and pupils may dilate or get large.
- They often can keep interacting with the people around them.
- Eyes may turn to one side and tonic-clonic movements may occur in some children.
- During a seizure, some children may be unable to see (lose vision temporarily), see flashing colorful or bright lights, or have blurry vision.
Seizures happen at any time during the day. Most often they happen during sleep or shortly after falling asleep.
Seizures tend to be prolonged, lasting 1 to 30 minutes. Longer seizures (lasting up to 2 hours) occur in 21-50% of children with PS. The longer the seizures last, the more likely they will progress to generalized tonic-clonic movements.
The seizure symptoms of PS are frequently mistaken as non-epileptic conditions, such as fainting, migraine, cyclic vomiting syndrome, motion sickness, sleep disorder, or stomach flu.
Is Panayiotopoulos syndrome inherited?
A minority of people (about 17%) have a family history of seizures or epilepsy. No specific genes have been definitively associated with PS.
How is Panayiotopoulos Syndrome diagnosed?
PS is diagnosed based on description of the seizures.
Others tests that may be done include the following.
The majority of children with PS have spike discharges on their EEG in many areas. These abnormalities seem on the EEG are mostly found in the occipital area of the brain.
- These spike discharges can shift slightly from one EEG to another. Spikes will often be more frequent when children close their eyes or are not fixating on an object, a phenomenon known as “fixation-off sensitivity.”
- Background activity is normal.
- An EEG with some sleep is important to obtain if possible.
MRI (magnetic resonance imaging)
The MRI, if done, is typically normal in children with PS.
An Example of an EEG in a Child with PS
How is Panayiotopoulos syndrome treated?
Seizures in children with PS are often infrequent and antiseizure medication may not be needed.
When seizures are more frequent, the seizures can be controlled with antiseizure medications like oxcarbazepine (Trileptal), carbamazepine (Tegretol or Carbatrol), levetiracetam (Keppra), gabapentin (Neurontin), zonisamide (Zonegran), lacosamide (Vimpat), and others.
Because children with PS often have long seizures, they may need emergency medical treatment. A rescue therapy may be helpful, such as diazepam rectal gel or another form of benzodiazepine given in the nose, under the tongue, or between the cheek and gum.
- 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.
What is the outlook for people with Panayiotopoulos syndrome?
Generally the outlook for children with this syndrome is good.
- Many children have few seizures during the course of the disease. Up to half of children have fewer than 4 seizures total.
- Children with PS will typically have normal physical and cognitive development despite the 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.
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