Panayiotopoulos Syndrome

Panayiotopoulos syndrome (PS) is a common form of epilepsy that is diagnosed in early childhood. It is also called early-onset childhood occipital epilepsy.1

PS is a benign form of epilepsy. Children with PS develop normally. The seizures caused by PS usually happen in the occipital lobe. This is the part of the brain at the back of your head.1

Symptoms of Panayiotopoulos syndrome

PS seizures begin as focal, affecting one area of the brain. Sometimes, a PS seizure will become generalized, affecting multiple areas of the brain. The symptoms of a PS seizure can be the same as in other disorders. For that reason, PS can be confused with other problems, like migraine or sleep disorders.1,2

PS affects the autonomic nervous system, which controls the body’s "rest and digest" functions. During a seizure, your child may:1,2

  • Look pale, become nauseous, or vomit
  • Pass out or have very dilated/large pupils
  • Have tonic-clonic movements: The tonic stage is when all the muscles stiffen, and the person may fall. The clonic stage is when their limbs jerk and twitch.
  • Lose their vision or see bright flashing lights
  • Complain of a headache after the seizure

PS seizures can happen at any time, but are most common while falling asleep or while asleep. They may also be triggered by fevers. The seizures can last up to 2 hours.1,2

What causes Panayiotopoulos syndrome?

Experts are not sure what causes PS. It may run in families, so experts believe that there may be a genetic cause. However, research has not found what genes may be involved. There may be an association between PS and febrile seizures (seizures that occur in children when their temperature spikes high).2

PS syndrome is thought to affect the occipital lobe of the brain. However, researchers believe that the autonomic symptoms are caused by unusual activity in the limbic system. The limbic system is a deep part of the brain that controls things like emotion and the "fight or flight" response.2

Who gets PS?

PS affects 6 percent of children with epilepsy between the ages of 1 and 15. It affects all sexes equally. PS is usually diagnosed between age 3 and age 6, but it can develop any time between 1 and 13.1

Diagnosis

PS can be diagnosed clinically, meaning that your child's doctor may be able to make a diagnosis just by hearing about the seizure symptoms. They can also use other testing to confirm, such as an electroencephalogram (EEG) or magnetic resonance imaging (MRI).1

An EEG measures brain activity. This can be done while your child is awake, but it may also be helpful while they are sleeping. Children with PS will show slightly different activity in the area of the occipital lobe. MRIs are not used to diagnose PS. Instead, your doctor will use the MRI findings to figure out whether other things are causing your child's symptoms.1

Treating Panayiotopoulos syndrome

Though PS seizures can last a long time, they usually do not happen often. Children living with PS may not need to take medicine. If they do, there are a few anti-seizure drugs available. Some common drugs are:1

  • Oxcarbazepine (Trileptal®)
  • Carbamazepine (Tegretol® or Carbatrol®)
  • Levetiracetam (Keppra®)
  • Gabapentin
  • Zonisamide (Zonegran®)
  • Lacosamide (Vimpat®)

Your child's doctor may also recommend rescue drugs. These drugs can be given as needed to stop a seizure that is lasting too long. Speak to your doctor if you think your child may need rescue drugs.1

The doctor will usually prescribe a benzodiazepine. This is a depressant drug, meaning it slows down the processes in your body. Your doctor will tell you exactly how to give the drug to your child in a way that is safe. Benzodiazepines come in the following forms:1,3

  • Rectal
  • Intranasal
  • Buccal (applied to the cheek and gums)

Outcomes

Children living with PS tend to have good outcomes. Many only have a few seizures and it is common for seizures to stop 2 to 3 years after they are diagnosed. Children with PS develop normally both physically and cognitively. PS does not increase the risk of seizures in adulthood.1

What should I do next?

If you believe your child may have PS, reach out to their pediatrician. They may refer you to a neurologist or an epilepsy specialist.

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Written by: Leah Steinberg | Last reviewed: August 2022