The Primary Care of Seizure Disorders: A Practical Guide to the Evaluation and Comprehensive Management of Seizure Disorders

Center.

PREFACE

This book is designed to help physicians who in the course of clinical practice care for patients who have epileptic seizures.

The intention is to provide a thorough but concise treatment of the subject which may serve as a basic text as well as a handy reference source for solving problems as they arise on a case-by-case basis. It is hoped that the tables and glossaries and the attention to the broader aspects of case management will be of particular value in this regard. The organization of the book reflects the sequence which clinical management often follows: differential diagnosis and genetic aspects, the electroencephalographic evaluation, medical therapy of seizure disorders including first aid and the treatment of status epilepticus, psychiatric aspects, problems of noncompliance and finding ancillary, community resources for patients.

The author would be pleased to hear from any reader who may have a suggestion for any future edition and would be grateful if notified of any error found in the text.

1

SEIZURE DISORDERS: SOME BASIC CONSIDERATIONS

Publisher Summary

This chapter provides an overview of different types of seizure disorders and some of the basic considerations. Statisticians have called epilepsy the hidden disease because it is believed that many people who suffer from this disorder are well-controlled and able to conceal their illness and therefore remain undetected by surveys. The prevalence rate for seizure disorders is no less than 3 per 1,000 populations and is probably much closer to 6–7 per 1,000. Approximately 85 percent of patients with seizure disorder achieve enough seizure control to lead essentially normal lives. Approximately half have 90–100 percent control. The extent to which anticonvulsant medication will be effective is usually known within the first year of treatment. Unemployment is always a major problem for those with epilepsy. The presence of epilepsy per se does not affect innate intelligence, and the majority of people with seizure disorders do not suffer intellectual impairment as a result of having seizures. The successful management of the patient who has a seizure disorder is a very gratifying experience for all concerned. It involves the cooperative effort of the primary care physician, the neurological specialist, the electroencephalographer, often the social worker, and the patient.

Statisticians have called epilepsy the hidden disease because it is believed that many people who suffer from this disorder are well controlled and able to conceal their illness and therefore remain undetected by surveys. Obtaining a random sample from the total epileptic population is nearly impossible. Bearing this in mind and weighing a great deal of evidence, the Professional Advisory Board of the Epilepsy Foundation of America has concluded that in the United States a minimum of 4 million persons suffer from some form of seizure disorder.

STATISTICS

The term prevalence refers to the ratio of those people affected by a particular problem to those not affected at a given time. The prevalence rate for seizure disorders is no less than 3 per 1,000 population and is probably much closer to 6 to 7 per 1,000. Incidence refers to the number of new cases within a population within a given period of time. The overall incidence of epilepsy is betwen 0.3 and 0.7 percent per year. In the United States the range of new cases is from about 23,000 to 147,000 per year. The Epilepsy Foundation of America points out that if a prevalence rate of 2 percent is assumed as constant the population growth alone between 1970 and 1980 should have resulted in approximately 70,000 new cases in the United States a year. This would reflect a 0.2 percent incidence rate. Epilepsy is a minor cause of death. Life insurance company estimates of death ascribable to epilepsy indicate a rate of 1.3 per 100,000 population. This is lower than the death rate from appendectomies.

Approximately 85 percent of patients with seizure disorders achieve enough seizure control to lead essentially normal lives. Approximately half have complete to 90 percent control. The extent to which anticonvulsant medication will be effective is usually known within the first year of treatment.

Unemployment has always been a major problem for those with epilepsy. Estimates of unemployment place the rate between 20 to 25 percent. When compared to other disabilities, seizure disorders have the highest rate of exclusion in hiring by virtually all firms surveyed. The outlook, however, is not all bad. The Epilepsy Foundation of America has estimated that there were as many as 1.7 million people with epilepsy in the 1972 labor force of 88 million. The three industries with the highest percentage of employees with epilepsy are banking, hospitals or clinics, and wholesale sales. The poorest opportunity for employment exists in government and private utilities.

INTELLIGENCE

The presence of epilepsy per se does not affect innate intelligence, and the majority of people with seizure disorders do not suffer intellectual impairment as a result of having seizures. Many famous and intelligent historical figures have had seizure disorders. However, an epileptic patient’s intellectual achievement is liable to the combined effects of social and psychological pressures, side effects of medication, and possibly unusually frequent and uncontrolled seizures. Of course some patients will have both seizures and intellectual impairment as a result of brain injury or progressive brain disease.

NEUROLOGICAL CONSULTATION

From the incidence and prevalence figures alone it is clear that physicians engaged in the delivery of primary care will have responsibility for both new and continuing care patients who have seizures. In many cases this is desirable simply because primary care physicians are frequently more available to patients than specialists.

The referral of a patient for neurological or neurosurgical consultation will depend to a large extent on the availability of specialists and to some extent on the community practices. Notwithstanding, it is useful to consider some specific reasons for referral. In order to provide some guidelines, the Departments of Family Medicine and of Neurology at Harbor UCLA Medical Center, Torrance, California, conducted a brief survey that included physicians in training and in practice. The responses focused on two main themes: (1) the proper use of medications and (2) the evaluation of seizures as part of the total neurological picture.

In patients who are being treated for epilepsy, the following may warrant consideration for consultation: poor control and unresponsiveness to usual medication doses, some intolerance or unacceptable side effect to usual medications, the management of medications during pregnancy, the problem of when to discontinue anticonvulsant medications, a change in some aspects of the patient’s seizure pattern, and the development of some new postictal symptoms or findings.

Neurological consultation should prove helpful for any patient who has a first seizure or seizures of new onset. The main questions to be answered concern the establishment or corroboration of the diagnosis and the extent of laboratory workup indicated. This is especially important when other specific neurological abnormalities have been identified. Consultation may also aid in the selection of anticonvulsant medication and the establishment of an effective maintenance dosage schedule. Two other reasons to obtain a consultation may be to comply with a patient’s or family’s request for a second opinion and to obtain genetic counseling.

The successful management of the patient who has a seizure disorder is a very gratifying experience for all concerned. It involves the cooperative effort of the primary care physician, the neurological specialist, the electroencephalographer, often the social worker, and always, of course, the patient.

REFERENCE

Epilepsy Foundation of America. Basic statistics on the epilepsies. Philadelphia: FA Davis, 1975.

2

THE CLINICAL EVALUATION OF SEIZURES

Publisher Summary

This chapter discusses the clinical evaluation of seizures. The great variability in the clinical manifestations of seizures may lead to imprecision in communication and disorderliness of thought in the differential diagnosis. Because of these factors, the international classification of epileptic seizures is recommended, which involves three major headings. (1) Partial seizures, refers to seizures that begin, and sometimes remain, in a focal and confined region of the brain. The clinical manifestations of such seizures will reflect the anatomical localization of function within the brain. (2) Generalized seizures, includes all seizure activity which is initially bilateral seizure activity that lacks focal or local onset. (3) Unclassified epileptic seizures because of incomplete data. The presence or absence of urinary incontinence is often cited as evidence for or against seizure activity. However, the occurrence of urinary incontinence is not a distinguishing feature of epileptic seizures. Conversion reactions with seizure-like activity are uncommon, and such a diagnosis usually requires formal psychiatric consultation for both diagnostic confirmation and successful management of the patient. The control of seizures has not been associated with prevention of mental retardation, a common concomitant of infantile spasms. Toxic-metabolic encephalopathies are common and often reversible causes of seizures, especially in hospitalized patients. Seizures caused by toxic-metabolic encephalopathies are often resistant to treatment with anticonvulsant medication. Febrile seizures are associated with an increased risk of intellectual deficit only in children who have a pre-existing neurological or developmental abnormality and who in addition have afebrile seizures.

The occurrence of seizure activity is a malfunction which is possible in all brains. Your patient, your patient’s pet, and even you, the reader, could have a seizure given the right circumstances. Whatever the factor that ultimately results in a seizure, the final common pathway involves abnormal function of excitable neuronal membranes. The kinds of circumstances that lead to seizures are, in fact, few and are readily identifiable.

A common and often reversible cause for seizures is a toxic-metabolic encephalopathy. The etiologies of toxic-metabolic encephalopathies, both endogenous and exogenous, are diverse and numerous. Electric shock and sudden mechanical force of the right intensity applied to the head may cause a seizure. Recurrent seizures may develop as a result of focal, pathologic processes involving the brain. Recurrent seizures may also occur because of an idiopathic and paroxysmal disturbance of brain function that is probably due to biochemical factors associated with brain inhibitory mechanisms.

The term epilepsy is usually employed to indicate those clinical cases in which seizures are recurrent and more or less unpredictable. The great variability in the clinical manifestations of seizures may lead to imprecision in communication and disorderliness of thought in the differential diagnosis. Because of these factors, it is desirable to have some scheme of classification of seizures in mind. The international classification of epileptic seizures is recommended for this purpose (Table 2.1). This classification has three major headings. The first, partial seizures, refers to seizures that begin, and sometimes remain, in a focal and confined region of the brain. The clinical manifestations of such seizures will reflect the anatomical localization of function within the brain. The next category, generalized seizures, includes all seizure activity which is initially bilateral seizure activity that lacks focal or local onset. The final heading is unclassified epileptic seizures (due to incomplete data).

Table 2.1

International classification of epileptic seizures

Abstracted from Epilepsia 11:102–113, 1970 and revised in 1978.

Even though seizures show variety, certain characteristics may be used to distinguish the epileptic seizure. No one of these characteristics, or hallmarks (Table 2.2), will stand alone, and exceptions may be found to most. However, taken in the aggregate they will usually allow one to make a decision as to whether the clinical event in question is a seizure or not.

Table 2.2

Hallmarks of epileptic seizures

Paroxysmal

Stereotyped

Alterations of consciousness

Brief

Reflect known neuroanatomical relationships

First, seizures are paroxysmal. They occur abruptly, with little or no warning, and interrupt normal, or at least ongoing, activity. This is true even when a person is asleep. When the seizure and any postictal disturbances are finished, ongoing baseline activity consisent with the patient’s former level of functioning is resumed. Complications of a seizure such as an aspiration pneumonia or injury, of course, may alter the outcome. An isolated seizure in and of itself has no lasting or deleterious effects.

Second, seizures are more or less stereotyped. Seizures in general have relatively uniform clinical patterns expressed by the terms grand mal, tonic-clonic, petit mal, absence, minor motor seizure, automatism (such as lip smacking in a complex partial seizure), focal motor seizure (rhythmical jerking of a body part), and other focal seizures. Most patients will usually have one or two seizure types which will show little variation. Observers quickly come to recognize the patient’s seizure as a personalized event with some minor variations on the main theme. The motor activity and behavior during a seizure are never premeditated or goal oriented in the sense that decisions are made and acted upon.

Third, some alteration of consciousness occurs with most seizures. In some instances this is a brief absence such as may occur in generalized epilepsy or in partial seizures with complex symptomatology (temporal lobe seizures). Or the alteration of consciousness may amount to little more than a confusional state during which there is imperfect recall of things that may have happened during the seizure. Often a short period of confusion or disorientation is present postictally. Rarely do seizures occur without any alteration of consciousness, and this is typified in a well-confined focal motor seizure. Any generalized tonic-clonic seizure always involves total loss of consciousness and total lack of recall for any events during the seizure.

Fourth, most seizures are relatively brief in duration. The time lapse is usually measured in seconds or minutes. Even in generalized tonic-clonic status epilepticus, the repetitive seizures usually last only a few minutes each; between the seizure is a quiet, postictal period from which the patient does not recover before the next seizure begins. Rarely, seizure activity may be prolonged over many hours or even a few days. Such instances have been recognized in complex partial status, spike and wave status (named for the characteristic electroencephalographic pattern), and focal motor status (epilepsia partialis continua). In both complex partial status and spike and wave status, the patient may principally appear to be in a confusional state. In complex partial status there may also be brief automatisms; in spike and wave status, there may be brief episodes of eyelid flutter or facial twitching associated with short periods of total absence.

Fifth, epileptic seizures reflect known neuroanatomical relationships. Focal seizures are expressed clinically by signs and symptoms that correspond to known functional localization in the brain. When seizures spread they may do so rapidly and result in a generalized tonic-clonic seizure which follows the focal seizure within a few seconds. By and large the term aura describes the focal onset of seizure activity which is the prelude to generalization. In some cases seizure activity will spread more slowly in anatomically contiguous areas. The classic example of this is the Jacksonian march during which seizure activity, for example, may begin in a thumb and then spread to the hand and face of the same side. Seizure activity may also spread to the opposite hemisphere via the intercerebral commissure. Seizure activity in the homologous brain region may be generated in this