General Principles of Management

The management of epilepsy can be conceived only as a global therapeutic strategy applied to individual patients. The management of the epilepsies of childhood has a wider scope than the sole prevention of both seizures and the other consequences of paroxysmal neurophysiologic dysfunction. The epilepsies are socially handicapping disorders, and even a single seizure occurring in certain circumstances may have disastrous effects. The impact of epilepsy on every aspect of both the child’s and the family’s life is considerable (Carpay et al., 1996, 1997; Hoare and Russel, 1995; Collings, 1990; Levin et al., 1988). Recurrent seizures severely disrupt the personal and social life of the patients, and uncontrolled epilepsy may definitively compromise both the physical safety and the overall quality of life of the affected children or adolescents. The risks of physical injury are real. Death, especially sudden death, has a higher incidence in the epileptic population than it does in the general population (Sperling et al., 1999; Nashef et al., 1995a, 1995b) (see Chapter 22).

The unpredictable character of the seizures represents a considerable burden in everyday life, and this applies even to those patients whose seizures are well controlled by drug therapy. Recent studies (Birbeck et al., 2002) have provided clear-cut evidence supporting the assertion that health-related quality-of-life improvement was consistently evident only among those patients achieving seizure freedom. The longterm outcome must also be considered, and appropriate advice on what the indications for treatment are must be sought (Sillanpää, 2000).

In addition to the obvious problems posed by overt seizures, apparent subclinical epileptic activity may, in some patients, profoundly disturb their mental functioning. This is seen in children with prolonged episodes of nonconvulsive status epilepticus. In addition, such an effect may possibly occur in those with major paroxysmal electroencephalographic (EEG) abnormalities that are unassociated with clinical seizures or that are accompanied only by infrequent and apparently minor attacks, such as those observed in the syndrome of continuous spike-wave paroxysms of slow sleep (Chapter 11); Landau-Kleffner syndrome; and the so-called atypical cases of rolandic epilepsy, despite the usually benign course of this syndrome (De Saint Martin et al., 2001; Massa et al., 2001).

Subclinical epileptic activity may have undesirable effects, at least in some patients. Several reports have indicated that isolated paroxysms or brief EEG bursts could interfere with ongoing cognitive processes (Aldenkamp et al., 1992; Kasteleijn-Nolst Trenité et al., 1988, 1990; Siebeling et al., 1988; Aarts et al., 1984). Such transient cognitive impairments may be global, especially with bilateral spike-wave discharges, which mostly affect the level of awareness, or more specialized for left or right hemisphere functions, as occurs in focal discharges (Kasteleijn-Nolst Trenité et al., 1988, 1990; Stores, 1971, 1980).

In addition, under certain circumstances, recurrent brief seizures may produce brain damage, and repeated stimulation by the frequent seizures and/or EEG discharges might, in itself, be conducive to an epileptic state. This is in concordance with the view that seizures are a self-perpetuating phenomenon (Shorvon, 1984). Such an effect could result from kindling (Goddard et al., 1969), but, more probably, it is due to different, as yet undefined mechanisms (Lado et al., 2002; Nehlig et al., 1999).

Therefore, all reasonable efforts should be made to prevent seizures and the other effects of epileptic activity. Treatment is clearly indicated in children with clinical seizures if the likelihood of recurrences is high (see Chapters 15 and 22). The indications for antiepileptic treatment for children with subclinical epileptic activity are more difficult to define. “Treatment of the EEG” is, in principle, not desirable in asymptomatic patients. Nonetheless, when cognitive difficulties or deterioration appear in a child with paroxysmal EEG discharges, treatment may be considered, even in the absence of classic seizures. At this time, however, no dogmatic statement can be made regarding this (Arzimanoglou, 2002b; Guerrini et al., 2002a). Children who are not initially treated for asymptomatic EEG discharges should receive regular neuropsychologic evaluation so that a slowly progressive decline of intellectual performances can be detected. In some cases, the interference of infraclinical discharges with mental functioning has been
clearly demonstrated to represent a major problem, and, after therapy was instituted, all difficulties were corrected. A librarian who was a patient of Aarts et al. (1984) had subclinical discharges that appeared only when he was concentrating on his work. When these were controlled, he was able to resume work efficiently. In patients with language disturbances (the Landau-Kleffner syndrome), these do not usually disappear as long as the ongoing EEG paroxysms remain uncontrolled, and controlling the continuous paroxysmal activity during sleep may result in an improvement in language function (Deuel and Lenn, 1997).

However, the control of seizures and especially of EEG paroxysmal activity should not be obtained by compromising the intellectual and physical capabilities of the patients. All antiepileptic drugs (AEDs) can have unfavorable effects on behavior and/or the learning processes (Aldenkamp, 2001; Trimble, 1990a; Aldenkamp et al., 1987, 1990), and heavy treatment may result in a severe deficit of attention and cognitive skills (Meador, 2001). The treatment must, therefore, remain compatible with a normal family and social life.

For many children, the sociopsychologic factors far outweigh the mere problem of seizure prevention. Epilepsy goes far beyond simply having fits, a concept that the clinician should keep in mind when he or she is planning comprehensive care for the individual patient. The aim of treatment is an improvement in the patient’s global quality of life. Control of seizures may be useless or it may even have a negative effect because epilepsy is a chronic illness that comes to dominate the life of patients and to constitute, in some cases, their pivotal interest and justification (Taylor, 1993; Trostle et al., 1989; Viberg et al., 1987; Betts, 1983).

TREATMENT OPTIONS

AEDs are the most commonly used therapy for epilepsy. All AEDs have some potential side effects, so the indications for their use should be carefully weighed as one of the main objectives is controlling seizures without producing drug-related undesirable effects. In the last decade, about 10 new AEDs have become available. However, the overall efficacy of both the old and the new drugs is moderate at best. In a recent study (Kwan and Brodie, 2000), only 331 individuals (63%) in a cohort of 525 patients were seizure free. The situation for infants and children is even more serious as a number of epilepsy syndromes remain difficult to treat. Adrenocorticotropic hormone and steroids can be used as antiepileptic agents for special indications, and they may be more effective than the usual AEDs (Deonna, 1991; Lerman et al., 1991; Marescaux et al., 1990). When the newer drugs are compared with conventional AEDS, the newer drugs are certainly tolerated better, but their potential side effects are not negligible (Marson et al., 1996).

Recent evidence suggests that various epilepsy syndromes respond differently to AEDs (Arzimanoglou, 2002b). Therefore, a more precise diagnosis and a better understanding of the mechanisms of action of AEDs enable physicians to use AEDs more specifically and more effectively (Aicardi and Arzimanoglou, 1996). The patient’s characteristics, including age at onset, seizure frequency, EEG data, and the findings of imaging studies, provide diagnostic pointers that permit the physician to reach a diagnosis of the syndrome and to choose the most appropriate AED for the individual patient. However, the specificity of AEDs is relatively limited, and, often, pharmacotherapy is begun with an agent with a broad spectrum of activity that is appropriate for the primary seizure type. Indeed, several antiepileptic agents have a similar or identical effectiveness for one particular form of epilepsy. Therefore, the eventual side effects and the difficulties in the practical handling of the drug play an essential role in the choice of a therapeutic agent. Cost must also be a consideration (Guerrini et al., 2001; Arzimanoglou et al., 1998)

The drug-induced exacerbation of seizures is a clinical problem that is often unrecognized or overlooked. Although further studies evaluating the prevalence of this phenomenon and investigating its mechanisms are required, a review of existing data (Guerrini et al., 1998c; Perucca et al., 1998) implicated the following two separate processes: (a) a nonspecific manifestation of drug intoxication due to excessive doses or polypharmacy or (b) a specific adverse action of a drug on specific seizure types or in certain epilepsy syndromes.

Carbamazepine has been reported to precipitate or to exacerbate a variety of seizures (myoclonic or absence), particularly in patients with idiopathic generalized epilepsies. In focal epilepsies, on the other hand, a recent report (Corda et al., 2001) suggests that aggravation by the drug is uncommon. Phenytoin and vigabatrin also have been implicated in the worsening of generalized seizures, whereas gabapentin has been associated with precipitating myoclonic jerks. Benzodiazepines have been reported to precipitate tonic seizures, especially in patients with Lennox-Gastaut syndrome (LGS) (see Chapter 24).

For some children, choosing not to treat the seizures may be appropriate. This no-treatment option may apply to provoked seizures, to those occurring in the context of an intercurrent disease, to almost all simple febrile seizures, to cases of rolandic epilepsy in whom seizures are rare, and to adolescents with isolated seizures (Camfield and Camfield, 2000; Wyllie, 1994).

Nondrug or nonconventional therapies include dietetic treatment, especially the ketogenic diet (Freeman et al., 1998; Prasad et al., 1996), and the use of immunoglobulins (Echenne et al., 1991; Illum et al., 1990; Arizumi et al., 1987).

Emphasis should be placed on the avoidance of stimuli that are capable of inducing seizures. According to Aird (1988), no fewer than 40 different stimuli can precipitate seizures, but the proportion of seizures that are induced by stimuli is poorly known (see Chapter 17). A range of figures from 5% to more than 50% has been given (Fenwick, 1992). These apply mainly to adults, and they include those seizures provoked by tense or stressful situations. In children, the roles of boredom and inattention as precipitants are well known (Olsson, 1990; Aird, 1988). Therefore, prohibiting many activities may indeed be counterproductive, and these children should be encouraged to lead an active life. According to Dahl et al. (1985, 1988), some children (12 of 18 of their patients) could identify situations in which the risk for seizure occurrence was low, and such knowledge should be used advantageously when it is available. These authors also found that some children were capable of identifying the onset of paroxysmal activity and of preventing the development of a seizure by countermaneuvers; Pritchard et al. (1985) found the same in 7 of 71 adults with complex partial seizures. Various forms of therapy, including self-control, relaxation, desensitization, or psychotherapy, have been used in an attempt to decrease seizure frequency (Fenwick, 1992).

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