Panayiotopoulos syndrome

25th March 2020

At present, it is not known how common panayiotopoulos syndrome is, it’s estimated that it could affect as many as 1 in 10 children with epilepsy but could also be closer to 1 in 20. Panayiotopoulos syndrome usually presents in children between the ages of 3 and 5, but has also been known to affect children as young as 1 and as old as 10. It is not believed to be a genetic syndrome that runs in the family and it affects both males and females equally. 

There are a number of other names used to describe panayiotopoulos syndrome, each of which is much longer, they are: 

  • Idiopathic susceptibility to early-onset benign childhood seizures with electroencephalogram occipital spikes
  • Self-limiting focal epilepsy of childhood with occipital paroxysms
  • Early-onset benign partial epilepsy with occipital paroxysms

Symptoms 

Children with panayiotopoulos syndrome will experience autonomic seizures, which are characterised by a sudden and unexpected change in behaviour. In addition, the child will likely become pale, nauseous and in most cases will vomit. Dilated pupils are a common feature of an autonomic seizure, alongside a flushed face, watering eyes and drooling, each of which are known as autonomic features. 

The vast majority of seizures (over two thirds) will occur during sleep. These can last long periods of time, sometimes around 20-30 minutes and as long as an hour in some cases. After a long seizure the child will start to feel their normal self quite quickly, although they may be upset at first. 

In terms of frequency, most children with panayiotopoulos syndrome will only have seizures once or twice a month. However, in some cases it can be more frequent. 

Diagnosis 

A detailed account of the child’s seizures are the key to a diagnosis of panayiotopoulos syndrome. A parent or guardian will likely describe the features listed above which will be recognised as autonomic. If possible, video recordings of seizures can be useful to aid diagnosis. 

An EEG test will likely be used to monitor for abnormal brain activity, which in this case is usually detected in the occipital lobes located in the back of the brain.  Interestingly, the EEG can return normal results in approximately 25% of children who have panayiotopoulos syndrome. Also Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) scans will usually fail to detect any abnormal brain activities, which can in itself  be a sign of the syndrome. 

Treatment 

Due to the infrequency of seizures, not every child diagnosed with panayiotopoulos syndrome will have anti-epileptic drugs (AEDs). Medication is usually only prescribed in cases where the child has more than 3 to 4 seizures and at the parents request. AEDs commonly prescribed include sodium valproate (Epilim), carbamazepine (Tegretol), Lamotrigine (Lamictal) and levetiracetam (Keppra).

Prognosis

The outlook for children with panayiotopoulos syndrome is very positive with most, if not all, children showing normal development and no learning or behavioural problems. The seizures themselves are not thought to be put the child at risk and in nearly all cases the child will stop having seizures within 2-3 years without treatment (also known as remission of epilepsy).  

For more information on panayiotopoulos syndrome, please contact us on 01706 373075 or email admin@nationalepilepsytraining.co.uk.