The Rett Syndrome Association of Ireland


A significant proportion of girls with Rett Syndrome will develop epilepsy. But it’s by no means inevitable, and it’s unpredictable. There are some features of Rett Syndrome that can resemble a seizure – breathing irregularities, twitches, spasms, head turning, laughing or screaming episodes – but are not. So the first thing to understand is that a Rett Girl may have epilepsy but may not, and how it presents is very individual. The information here is offered is no more than a basic introduction to a very complex issue.

What is Epilepsy

Epilepsy is a neurological condition caused when sudden changes in the brain’s electrical balance trigger seizures. To be considered as epilepsy there must be recurring seizures, as it’s not uncommon for people to have a seizure as part of other conditions. In fact, about 1 in 20 people will have a seizure at some point in their life.

Electrical activity is part of the normal working of the brain. It’s the way that the cells in the brain communicate with each other and the rest of the body. Neurons send electrical impulses to surrounding cells and they, in turn, “fire” electrical impulses to cells adjoining them. During a seizure too many neurons fire at the one time, causing a sort of “electrical storm” in the brain.

What is a Seizure

seizure is a “change in sensation, awareness and behaviour” brought about by this “electrical storm” in the brain. There are up to 20 different kinds of seizures, they can last from a few seconds to a few hours (but those are very rare cases).

Considering the broadest terms seizures can be classified based on where they occur within the brain. Broad headings includegeneralised and partial seizures, there are also status epilepticus. and non-epileptic seizures

Generalised seizures occur when electrical abnormality (or “electrical storm”) occurs throughout the brain. Principal types include

  • Grand Mal – the person will collapse, lose consciousness, the body stiffens with violent jerking, usually followed by a deep sleep.
  • Absence – the person experiences a brief loss of consciousness, which lasts only for a few seconds. Often the patient interrupts an activity and stares blankly. They start and end abruptly, and can occur several times a day. The person may not even be aware of the event
  • Myoclonic – the person experiences sporadic jerks, usually on both sides of the body. often described as like electrical shocks.
  • Clonic – rhythmic and repetitive jerks in both sides of the body at the same time.
  • Tonic – marked stiffening of the muscles
  • Atonic – in contrast to Tonic, a sudden loss of muscle tone, especially in the arms and legs.

Partial Seizures (sometimes called focal seizures) occur when the electrical event is limited to only a part of the brain. There are two main kinds:

  • Simple Partial A person will conscious but they can experience a range of unusual sensations: jerky movements or twitches of a body part, distortions in hearing or seeing, stomach discomfort, or a sudden sense of fear. It’s commonly thought that the “aura” described by some sufferers is actually a partial seizure.
  • Complex partial seizures can lead to lost or altered consciousness. A person might stare at nothing, lose the ability to control body movements, have trouble speaking or responding. Afterwards the person might not remember it happened.

Status epilepticus is when someone has continuous seizures with no recovery between events. If the seizures are convulsive then this is a very serious condition, possibly life-threatening. Medical help should be sought immediately. As a general rule a tonic clonic seizure lasting longer than five minutes, or a seizure lasting more than a couple of minutes longer than a person’s usual pattern, can be considered reason to contact the Emergency Services.

The two major categories of Non-Epileptic seizures. include

Physiological Seizures (caused by a variety of conditions that can be the result of a sudden change of blood supply, sugar or oxygen in the brain. These include changes in heart rhythm, sudden drops in blood pressure or hypoglycemia.) and PhyschogenicSeizures (caused by stressful psychological experiences of emotional trauma, and often indicate the body is under excessive stress.) Even binge drinking and alcohol withdrawal can trigger seizure events.

Managing Epilepsy

Epilepsy cannot be cured but it can often be managed with any of a number of drugs, sometimes used in combination, sometimes on their own. Effective medication will not stop seizures but it can help minimise the number and the severity of seizures. Different drugs can be effective against different kinds of seizures. It’s important to stick to the medication plan worked out with the Doctor. Sometimes it can be possible to identify triggers, or situations which make seizures more likely. If you can identify such a situation it’s a very good idea to avoid them.

What to do if your child has a seizure?

Experienced parents will have a protocol for managing during a seizure, so the information here is aimed at someone who is dealing with the onset of seizures, and may not even be sure of what they are witnessing. A seizure can be a frightening thing to witness especially if you do not know what to do.

Once a seizure begins there really is little to do until it ends. You cannot even do basic CPR because the chest won’t expand. These few Does and Don’ts are a very basic guide:

  • Don’t Panic Most seizures are quite brief and end of their own accord.
  • Safe Area Ensure that there is nothing around the person that they can hurt themselves against – tables, chairs, cup, glasses etc. If someone is standing at onset, try guide them to the floor gently. If possible try and put a cushion under their head.
  • Note the time It can be useful to know how long an episode lasts. Try and note the sequence of events as they unfold. This information can be very useful in making a medical assessment. If possible, a video of the event – even shot on a mobile phone – can be useful for diagnosis.
  • Do Nothing Don’t restrain the person, don’t try and hold them down or limit their movement.
  • Under No Circumstances put anything into their mouth – especially your own fingers. It’s a common concern that someone seizing may swallow or bite their tongue during a seizure. While this might occur in very rare cases, putting your fingers into their mouth can result in damage to jaws or broken teeth for them and very badly bitten fingers for you.
  • If you can try lie them on their side in the Recovery Position. Loosen tight clothing.

After a seizure it’s very likely that the person will sleep. If they have turned blue during the seizure then they will recover their own colour over time. Stay with the person until they are awake and alert, be comforting and reassuring.

If this is the first seizure that your child has had, then you will now need to contact your Doctor.

Some links that may be of interest:

Brainwave Ireland

The Epilepsy Foundation

Seizure First Aid

This article is published here for information purposes only. Medical advice should be sought from a Medical Professional.