Panayiotopoulos syndrome
Epilepsy Action is indebted to Dr Richard Appleton, a Consultant Paediatric Neurologist who specialises in children’s epilepsy, and to Dr Rachel Kneen, Consultant Paediatric Neurologist and Dr Stewart Macleod, Specialist Registrar in paediatric neurology, at Alder Hey at Alder Hey Children’s Hospital, Liverpool, who have kindly prepared the information on this page.
This is also known as:
- early-onset benign partial epilepsy with occipital paroxysms
idiopathic susceptibility to early-onset benign childhood seizures with electroencephalographic (EEG) occipital spikes.
This is a recently described epilepsy syndrome. It is said to be a relatively common type of epilepsy affecting about one in seven or one in 10 of all children with epilepsy. The most common age it begins is three to five years but it may affect children aged as young as one, and as old as 15 years of age.
Boys and girls appear to be equally affected. It does not usually run in families. However, more research needs to be undertaken in this epilepsy syndrome.
Early-onset benign partial epilepsy with occipital paroxysms (Panayiotopoulos syndrome) is different from the epilepsy syndrome called ‘benign epilepsy of childhood with occipital paroxysms (BECOP)’. In this other syndrome (BECOP), the children are usually older and the seizures consist of problems with vision and flashing lights and are usually accompanied by headaches.
Symptoms
The main seizure type is known as ‘autonomic’. The child may have a sudden change in behaviour and then they become pale, complain of feeling sick and will usually vomit. The pupils of the eyes often become very large (dilated) and there may be a lot of sweating and drooling (these are the ‘autonomic’ features).
The child may become unresponsive and their head may turn and become fixed to one side. It may remain turned to the side for many minutes. Very often the seizure will then end by jerking movements (called clonic movements) down one, or occasionally both, sides of the body. After the seizures the child will be upset and may sleep.
Over two thirds of the seizures will occur in sleep; the sleep may be during the night or during a day-time nap. The seizures are often long-lasting and may last 20, 30 or even 60 minutes. Even following the long seizures, and after the child has woken up, they will be back to normal.
The seizures are very infrequent. Many children will have just one or two seizures while others may have four or more.
Diagnosis
The diagnosis of early-onset benign partial epilepsy with occipital paroxysms or Panayiotopoulos syndrome is made on a detailed account of the child’s symptoms.
Important features in making the diagnosis are: the episodes usually occur during sleep and are nearly always accompanied by autonomic features and vomiting. The child’s age is also important, as most seizures occur in young children.
The electroencephalogram (EEG) often shows abnormal activity (called spikes) on both sides of the brain and usually towards the back of the head (in the part of the brain called the occipital lobes).
Sometimes the EEG may only show the abnormality if it is carried out when the child is sleeping. However, the EEG may also be normal in up to a quarter of children with this epilepsy syndrome.
Brain scans (CT and MRI) are usually normal.
Treatment
Not every child needs to be given anti-epileptic drugs. This is because the seizures occur infrequently and because most children will stop having seizures soon after the seizures start.
An anti-epileptic drug is usually prescribed if the child has had more than three or four seizures and particularly if the parents are worried.
The anti-epileptic drugs most commonly used are sodium valproate (Epilim) or carbamazepine (Tegretol); the newer drugs may also be effective, but this is not known for sure.
Prognosis (outlook)
Most, if not all children with early-onset benign partial epilepsy with occipital paroxysms or Panayiotopoulos syndrome, will show normal development in the first few years of life. Most will also have no learning or behaviour problems. Even the longer seizures do not cause any problems and it is not thought that the seizures are dangerous.
Nearly all children will stop having seizures within two or three years after the seizures first started. This is called a ‘remission’ of the epilepsy. The remission of the epilepsy is spontaneous (it happens of its own accord) and is not affected by the use of anti-epileptic drugs.
Finally, it seems to be very rare for a child with this type of epilepsy to have seizures either continuing into, or re-starting, in adult life.
Support organisation
Contact a Family, 209-211 City Road, London, EC1V 1JN, telephone 0808 808 3555, http://www.cafamily.org.uk/
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Comments
My 4 year old daughter was recently diagnosed with epilepsy after having seizures each lasting approx 30 mins and we have just been told she suffers from panayiotopoulos syndrome. After reading this article, I can see that everythings fits into place. She always has seizures (as described above) whilst in bed but on at least 2 occasions she has managed to get our attention (thank goodness for baby monitors!)
She has been on carbamaepine for 3 months or so and has only had 1 seizure during that time. Her consultant seems to think this ocurred because she was on antibiotics at the time which may have affected the absorbancy of her carbamazepine.
Seeing this article about the syndrome has made me feel more positive about her condition
my daughter aged 7 was diagnosed with panayiotopoulus epilepsy today. she had s very bad seizure 7 months ago whilst on holiday, it was a long seizure and she ended up in itu, this is the only one she has ever had, the consultant want to begin medication, but not sure if this is best?
very confused about whole situation
Just a seven year old boy when it happened. There was no explanation for this ordeal. A weekly shopping trip with the parents, to our local Somerfield with the overly priced bill waiting to take a chunk of mum’s purse before the exit doors; I thought to myself, will I get away with the Dr Pepper drink that I had so obviously buried beneath one of our more familiar buys, Walkers Crisps?
After Dad had driven us home, both my parents, as they usually would, took the shopping from the car and packed it into our exceedingly organised cupboards, meanwhile I made a sprint for the TV remote, as I knew that once I had started watching my programme mum wouldn’t have bothered trying to fight about watching hers. As the standby switch turned from red to green like the traffic lights at a drag race, it didn’t just activate the telly. It ignited Panayiotopoulos! (Later I was to discover this is a form of Epilepsy that would haunt me for years to come).
I had never even seen anyone experience anything like this, let alone experience it myself; these unforgiving and relentless lights controlled my eyes, beating and thumping, driving me into hypnosis, forcing my pupils to pursue them. The sensation was incomparable. The only way I am able to compare this force is to say that it felt as if my eyes were being used as drums in one of the longest concerts of all time and instead of using drum sticks the drummer was using sledge hammers. They were and still are, the only lights that I have ever seen that are as bright with your eyes closed as when your eyes are open. With my pupils beginning to dilate I asked my parents if they could see the same thing that I could, but for them there was nothing, this was the most menacing factor as it was the only moment to this day in which neither of my parents could understand me!
These vigorous lights glazed my vision for the next 15 minutes before the projectile vomiting began. Nothing that I or anyone else could do would have stopped me at that moment in time. I threw up and threw up until my stomach was forcing every fragment of life up through my oesophagus and into the dark orange bowl that mum had been holding to rescue the carpet. I’ve never had such a strong feeling as the one that I was about to encounter. My eyes were slowly but surely beginning to close and at this point I knew I was in danger, it felt like a Disney film without the fairytale ending, and in this Disney the good guy wouldn’t be saved.
My eyes, along with my entire living system, had closed down, with no life within it, and the only exceptions were an odd powerful push of desperation. Mum who was and still is a nurse dialled 999 immediately after realising something was wrong, but I myself was unable to hold consciousness until the ambulance arrived. What occurred next could only be explained to me by my parents when I awoke in the high dependency unit in hospital surrounded by many doctors, nurses and my pale faced parents asking how I was feeling, whilst looking at all the wires and tubes I had attached to me. I was later informed that I had had an enormous fit that had lasted for ninety minutes. I had raced at high-speeds in the ambulance to hospital with a doctor following behind in his car, and my parents having been told I was very ill and that I may not pull through, Even to this day I cannot imagine how frightening that experience must have been for them, especially when they are incredibly over protective of me anyway, and how frenzied it must have driven them watching me as I fit and wandering if I was going to live, knowing that they could now do nothing to save me.
Despite that traumatic experience life went back to normal as quick as possible and I was allowed to be a ‘normal’ young boy, it never stopped me from being told off when I was doing something wrong or being told to do things I was supposed to be doing (such as homework). I would regularly attend outpatients’ appointments and have EEG’s. My parents did some research and through the help of a homeopathic Dr also tried things such as diet (Yuk!) no sugar for a number of years, yet the fitting continued following a similar pattern of seeing flashing lights, having pains in my head, vomiting and then fitting for long periods of time, being rushed into hospital and ending up in resuscitation until my condition stabilised.
Despite this nobody could tell me exactly what was wrong, both I and my parents searched for answers through expensive visits to homeopathic doctors, gaining knowledge from epilepsy support groups/help lines and much research through the internet and library. Yet nothing I experienced seemed to be explained in any previous case study or article about Epilepsy.
One of my strongest memories of this time was my parents almost arguing with the consultant and refusing to accept that the specialists medicate me without finding what the cause was and whether the treatment was the best solution to the problem this seemed to cause the specialist somewhat of a dilemma as they could not find the diagnosis. Then one day whilst undergoing one of my EEG’s the technician started to ask some questions about my experiences, initially I thought he was being nosy but as he asked the questions he seemed to understand my symptoms it was then he explained that he had just been on a course about Panayiotopoulos syndrome, (a rare form of childhood epilepsy) and from there we were able to research the condition, find the best treatment and talk to the consultant.
It is only now that I can appreciate how right my parents were to challenge the ‘experts’ and the effort and pressure it took, to not just accept the ‘experts knew best’, however as a young child I remember feeling it was just my mum being bossy as normal because of her job as a nurse. My outpatients’ appointments became quite comical. I knew it would lead to my mum challenging the consultant and his team; they often looked uncomfortable and would compromise on my treatment. As a child this was fun to watch and later in life gave me confidence to question people about the treatment they suggested.
After people knew I had Epilepsy, some people behaved differently towards me, some parents of friends of mine would no longer ask me to tea for fear that I might become unwell, some teachers would be fearful of not being trained to deal with me should I have a fit this would make me feel frustrated and angry as to me I was ‘normal’.. Luckily my friends treated me exactly the same as they always had, playing as normal and even when I did start to see flashing lights in the playground it was my friends who acted more grown up than the grown-ups themselves guiding me to an adult teacher who could then phone for an ambulance, besides it gave us all something to talk about when I returned to school. After all it was not everyday an ambulance drives into the school playground.
Then a change in sequence occurred, Man united vs. Liverpool mum had gone to a fancy restaurant with her work mates, leaving dad and I watching the footy, after a thrilling first half and what could potentially become the game of the season turned into an experience I would never forget, but this one certainly wasn’t positive. A long ball up field slowly but categorically turned my transfixed pupils into a blur and then; total darkness I could see nothing. I was blind!
I informed dad immediately, who then sat me down and persuaded me that it would be fine, thankfully he was right after no less than half an hour of total darkness my vision was restored and I don’t think I have ever been more thankful in my existence than on that Wednesday evening.
There were also some experiences that I can now look back at and laugh at, in particular there was a time when my mum, cousin and I were returning from town, my mum was driving it was very busy and we were stuck in a traffic jam, my cousin and I were in the back of the car, all of a sudden I see flashing lights and whilst mum was panicking and trying to get across the traffic she told my cousin to get something from the back of the car to be sick in. My caring cousin then passes me a cushion,(what he thought I was going to do with that) to this day I will never know, again leading to yet another trip to hospital in an ambulance, however my cousin was very excited as he got to ride in the front of the ambulance.
It was all somewhat exciting at that age and once the trauma was over everything quickly reverted back to normal, as the years went by and I was growing up my image became more important to me and for some time talking about my condition was not acceptable to me especially seeing I was going to senior school with Panayiotopoulos syndrome, which was incredibly daunting for a while and took away the excitement of starting a new school. Yet with careful negotiation and planning the transition was the best thing that ever happened to me as I made a lot of friends who remain friends to this day; And although they have seen me fit, they don’t treat me any different to anyone else (in fact I don’t get any special treatment at all).
To date I still have regular outpatients appointments in which being so large in comparison to all the other kids on the children’s ward, so much so that I am to tall for them to measure my height and too heavy for their regular scales.
When in the, once daunting now familiar Derriford Hospital that I’ve grown in parallel to; I sometimes wander what I ever did to deserve such a disabling illness, yet can see it is like any other illness and is only as disabling as I let it be, even after being informed by my consultant that I will never join the armed forces or acquire a HGV licence, I am awaiting my 17th birthday on which I will then be able to drive after going more than 12 months without a seizure and by doing so I have realised that there is nothing I cannot achieve or nothing I cannot accomplish.