Educationals

What should the patient, his family and his school know; what can't he do?

Epilepsy is probably the most stigmatizing disease and parents and families go to extraordinary lengths to conceal this from their family and even the school authorities. Though it is easy to make a blanket statement that this is just another disease and everybody should be informed it is sometimes difficult to convince parents of this. What the author does is to inform the patient what epilepsy is and reassure them of the benign nature and outcome of most epilepsies starting in childhood. At every visit it is stressed that epilepsy is a just another disease and nothing to be ashamed of. Once patients understand how common this is, they tend to treat it just like other diseases. Samman is an epilepsy support group in Mumbai that helps this process of
acceptance.

Who should know? We feel this should be handled on `a need to know' basis. Obviously school and class teachers must know, as they should not be faced with an unexpected seizure. We even encourage the parents to educate the teacher on first aid measures and some even keep the rectal DZ at school. Neighbours, family members and friends where the child spends substantial
time must also be informed. We feel that as the accident rate in epilepsy is quite low (probably epileptics and their parents are reflexly more careful), it is better to supervise activities like swimming, cycling etc, rather than impose strict restrictions. Girls should stay away from kitchen stoves. The patient should not feel deprived. Downplaying the parents' anxieties and avoiding overprotection is also beneficial, as this leads to secondary anxiety and overdependance in the patient. Toddlers with drop attacks should wear cycle helmets to prevent injury. This supervision should continue for at least a year after the seizures are in remission.

Discontinuation of therapy

In epidemiological studies approximately 70-80% of patients enter long term remission. From developing countries data on untreated patients also suggests that at least 50% enter long-term remission. Hence it appears that the majority of patients will lose their epileptic tendency regardless of treatment. AED therapy is probably only symptomatic, preventing recurrent clinical seizures and having little or no effect on the natural history of epilepsy. If the epileptic tendency spontaneously resolves, AED therapy will not be needed and should be discontinued. This helps not only as the patient is spared the myriad adverse effects, but also as the patient and families feel a sense of relief that they are past the disease.

Several studies have addressed the risk of relapse after discontinuation to try and establish a predictive model that might help both patient and doctor to decide whether the risk is worth taking.

The risk of relapse ranges from between 20-40% in most studies and this risk is maximum in the first year after discontinuation. About 75-80% of the risk is in the first year and this is almost completed by the end of the fourth year suggesting that almost all patients who cross the four year mark will probably not have a recurrence and can be considered `cured' of their disease. The
median risk at 1 year is about 25% and by the second year about 29%. Several factors have been studied to decide which are most predictive. These will be discussed in some detail.

Age at onset

Remote symptomatic seizures are those that are symptomatic of brain injury sustained sometime in the past. Clearly the risk is increased about one and a half times in these children, especially those with mental retardation of a severe degree. Cerebral palsy does confer some risk though this is much lower.

An abnormal EEG at discharge probably increases risk of relapse as well; exceptions are the benign partial epilepsies where there is little correlation of an abnormal EEG and clinical seizure occurrence. Focal slowing on the EEG and epileptiform activity are probably important risk factors.

Electroclinical syndromes if diagnosed, sometimes help, as studies have shown that BECTS always remits, JME rarely remits while BEOPS and absence epilepsy remit 75-80% of the time. The problem is that syndromes are diagnosed in less than half the children.

Type of seizure does not really help as similar seizure types can occur in and malignant epileptic syndromes.

Severity of epilepsy i.e. a longer period of active epilepsy, higher number of seizures prior to stopping AEDs, etc probably confer an additional risk though this has not been studied in a systematic manner. Status epilepticus is probably not a major risk factor.

Other equivocal risk factors include breakthrough seizures during fever in children. Family history does not seem to be a major risk factor; neither does a previously failed attempt at discontinuation, suggesting that one may try this process more than once.

Even those who are seizure free on AEDs have a 20% risk of relapse suggesting that this is part of the natural history of epilepsy which AEDs do not apparently influence.

Generally the longer you are seizure free the higher chance of remission. The minimum seizure free period would be about two years, though studies with 1 year and 5 years of seizure freedom have shown similar rates of relapse. These years of seizure freedom probably are not important in themselves and these figures probably reflect on the substrate of the particular epilepsy syndrome.

There have been studies to try and correlate the number of risk factors with the risk of relapse and have found that with no risk factor, relapse rates are as low as 12%, with one risk factor this rises to 46% and with two factors to 71%. These figures are similar for cryptogenic and remote symptomatic seizure disorders.

Finally the patient or family must understand all these risks and they should be encouraged to participate in the decision making process. It is always a good idea to try discontinuation before driving is learnt and before the child becomes totally independent and unsupervised. The risk of injury and status epilepticus, though low, is obviously more of a problem in an unsupervised teenager who keeps late hours, misses sleep and may drink alcohol.

For one year cycling on the road, unsupervised activities like swimming, cooking etc should be avoided, as the maximum risk of relapse is during this period.

Tapering of drugs over 6-8 weeks is adequate and long drawn out tapering periods do not decrease the risk of seizures, withdrawal seizures are a problem more with PB, PM, BZDs and VGB. If a patient does relapse most will need a reinstitution of treatment though one could wait if an obvious precipitating factor was involved.

In summary, the average risk of relapse after discontinuation of AEDs after a two-year seizure free period is about 30%. This risk is higher if the age at onset was in infancy or adolescence, in those with remote symptomatic seizures and in those with abnormal EEGs at time of discontinuation. Certain electroclinical syndromes also determine prognosis of relapse. Severity of epilepsy, duration of active epilepsy, difficulties in achieving control and breakthrough seizures with fever, are probably risk factors as well. A full discussion with the family about the risks and benefits of discontinuation is very important as the child and parents should feel part of the decision making and must understand that they may need AEDs again. Short tapering over 2 months is adequate. The biggest advantage of discontinuation is the feeling of well being which occurs as the child and the family feels that they are rid of the disease.

This review of the salient points of management of epilepsy is far from complete. A complete list of references and suggesting reading is with the author and available on request.