Behavioral Therapies
Peter Wolf
To abolish this disease, one must apply the element which is opposed to it, and not what is favourable and habituated.
On the Sacred Disease—Hippocrates
Introduction
Epilepsy is traditionally treated with drugs, and, for pharmacoresistant cases, there may be a possibility of epilepsy surgery. But, although little noticed in textbooks, there is a third line of therapy that can be useful if applied to the appropriate patients: Behavior modification. Behavior modification can be defined as any type of nonpharmacologic intervention aimed at the prevention or abortion of seizures. Like all other epilepsy therapies, the ultimate goal of behavior modification is complete seizure control. To achieve this goal, behavior modification can be used alone or in conjunction with other therapies. It can be used to arrest seizure activity after onset or to prevent seizures altogether.
Arrest of Seizures
History
Treating seizures by some countermeasure that interrupts their development is the oldest of all existing therapies. Probably discovered by patients, arresting seizures was mentioned as an established procedure by Hellenistic physicians such as Aretaeus the Cappadocian and Galen, in the first and second centuries. It was well known to physicians that seizures beginning distally in a limb and spreading to the brain could be interrupted by manipulations such as the application of ligatures. This may have contributed to the concept of sympathetic epilepsy, which was thought to originate in the periphery where a pneumatic substance would spread through the body and to the brain like poison from the bite of a scorpion.30 The ligature was applied in view of arresting the spread of this substance.
The arrest of seizures through application of ligatures remained in use throughout the history of medicine. Tissot31 mentions numerous examples and regarded the effectiveness of such procedures as supporting the concept of sympathetic epilepsy. One of the discoveries that seriously shattered this concept was the finding of Odier in 181111 that ligature arrested fits due to a cerebral tumor as effectually and more frequently than with any other cause.
Under the influence of Brown-Séquard’s experiments, where external stimuli produced convulsions, Jackson15 suggested in 1870 that a similar relation existed between a peripheral aura sensation preceding the seizure and the internal discharge, the beginning of which was reflected in the outward spasm. However, such a relation could explain only the action of a ligature applied between a first aura sensation and the onset of spasm—not after the onset of the spasm.
Gowers11 conceived a totally new theory:
“The arrest must be effected, not in the limb convulsed, but in the centre in which the discharge is occurring, of which the local convulsion is the outward manifestation. The strong peripheral impression on the limb above the part convulsed probably raises the resistance in the nerve-cells of the corresponding parts of the brain, and thus arrests the spread of the discharge. The effect of the ligature must first be exerted on the sensory centre, and through this on the motor centre. The two are connected intimately, and their mutual interaction must be constant, so that the condition of the motor centre is no doubt readily influenced through the sensory centre. Hence, a fit may be arrested by the ligature, whether it begins by a sensation or by a motion, i.e., whether the sensory or motor centre leads in the discharge. Additional observation is necessary to determine whether the ligature is more effective in one class of fits than in the other.”
The knowledge and discussion of these phenomena were common to the British neurologists of the late 19th century. The Jacksonian seizures of the first patient operated on for epilepsy without evidence of a lesion13 began with flexor twitching of the thumb, and were arrested by stretching the thumb or applying a ligature. For Horsley, this suggested “the possibility of the so-called muscular sense being represented in the excitomotor area.”13 Ligature and passive stretching of a muscle in spasm are simple countermeasures that have often been reported. In other instances, much more intricate procedures were applied.15
Gowers11 reported a variety of countermeasures (Table 1). Ligature proximal to convulsion, forcible prevention of spasm, and other cutaneous stimulations were often successful in seizures commencing in a limb. Muscular exertion, strong olfactory impression, and strong gustatory sensation were sporadically effective in “attacks which begin by a general or bilateral aura, or by the epigastric sensation” and which can rarely be arrested. However, Gowers pointed out that nitrate of amyl, which had been given in view of vasal dilation, was most successful in cases with a deliberate olfactory aura, perhaps acting through the olfactory nerve.
At the turn of the 19th century, when bromides were the only effective pharmacotherapy available whereas epilepsy surgery mostly dealt with focal motor seizures and carried a rather high mortality risk, treatment by behavioral seizure arrest was an important and sophisticated part of the therapeutics of epilepsy. The advent of new, more potent, and better tolerated antiepileptic drugs starting in 1912 with phenobarbital changed this. Epilepsy came to be considered as a disease for the most part curable with drugs that make the seizures disappear.
In more recent years we have become aware that not all patients respond sufficiently to the available drugs, nor do all patients tolerate all drugs well. New drugs have new side effects and are not effective in all pharmacoresistant patients. Epilepsy surgery for these patients is by no means always possible. Stimulation methods are still experimental or have the drawback of being invasive but only palliative.
Table 1 Arrest of Seizures | ||
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Therefore, additional therapeutic options are sometimes most welcome. In addition, quite some patients today hesitate to take drugs, especially on a long-term basis, and ask for treatment alternatives. Often, such patients are prepared to accept responsibility for and contribute to a therapeutic strategy. Ketogenic and other diets are one type of nonpharmacologic conservative intervention and are dealt with in Section V, Part E, Alternative Approaches. Behavioral modification is another.
Modern Accounts
The modern literature about treatment with seizure arrest starts in 1956 with the important case report of Efron7,8 about a patient whose epilepsy became cured by using an olfactory stimulus to arrest her habitual olfactory aura. No drug treatment was involved. The olfactory stimulus had been the patient’s idea, and it took some time to find the most effective smell and the correct timing of the stimulus. The second and even more interesting step in this case report was the conditioning of the olfactory stimulus to a visual stimulus following a request by the patient who, being a professional singer, wanted to avoid the visible procedure of using a sniff bottle in public. She learned to evoke the smell by looking at and, later, thinking of a bracelet that was used as the conditioned stimulus. After some time, the patient began to experience the conditioned smell instead of her habitual aura; eventually this also stopped.
For a long period, no replications of Efron’s work were reported, but two authors22,23 found independently that about 60% of patients with localization-related seizures had personal experiences with countermeasures to arrest ongoing seizure activity. Richard and Reiter24 estimated that practically all patients had this kind of experience, but this statement is not based on specific investigations.
The applied countermeasures belong to a variety of categories. Specific sensory stimuli, such as a peripheral ligature for focal seizures commencing in a limb or an olfactory stimulus in a seizure with a habitual olfactory hallucination, relate directly to the cortical area involved in the seizure generation and spread. Many patients apply nonspecific arousal and concentration techniques,24 the action of which can be understood by the well-known antiepileptic effect of exercise17; others rely on relaxation procedures. The antiepileptic actions of yoga41 and of aromatherapy1 have recently been reviewed.
Anecdotal Observations
Chance observations of patients who are not in medical treatment have led us to assume that successful self-therapy by seizure arrest may not be uncommon among patients who are not undergoing medical treatment. Many of these patients may not even be aware that they are dealing with epileptic phenomena.36 This possibility should be considered in future epidemiologic field studies of epilepsy because there is no other way of quantifying it.
Table 2 Curative Effect of Aura Interruption: A Hypothesis | ||
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Our own experiences with seizure arrest in drug-resistant patients in a specialized epilepsy center by methods building on spontaneous patient experiences and cortical direct-current (DC) biofeedback6 indicate that, in resistant patients, treatment by systematic seizure arrest is neither less nor more effective than is treatment by a new drug.36 However, successful behavioral therapy is only possible with well-motivated patients because it requires an unusual amount of effort and compliance. Sometimes, the intervention must be supported by comprehensive psychotherapy.2 When patients are successful, increased self-esteem and improved self-control are welcome side effects.
We do not propose that patients use trained seizure arrest as a treatment alternative but, rather, as part of a comprehensive therapeutic program that includes pharmacotherapy. In some patients, a tripartite approach combining pharmacotherapy, surgery, and behavioral seizure arrest may be most appropriate.
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