Classification Clinical Manifestations and Pathology

The growing emphasis on physiological mechanisms and electroclinical correlations has led to a classification of the epilepsies by the localization of the electrical abnormality in the brain. The major division is between generalized (centrencephalic) seizures, where the brain activity is spread over the entire cerebral cortex; and partial (focal) seizures, which occur when only one part of the brain is involved. Generalized seizures demonstrate bilateral motor activity and involve a loss of consciousness, which may or may not occur in partial seizures depending upon the part of the brain initially affected and the subsequent involvement of other structures. There is an approximate correspondence between the sites of the brain where electrical abnormality occurs and the clinical manifestation of the seizure.

Generalized Seizures

The term "epilepsy" was first used to denote the symptoms of major, or grand mal seizure, currently referred to as a tonic-clonic seizure. To this day, over 60 percent of all individuals diagnosed as epileptic have tonic-clonic seizures. A sudden burst of discharges involving the whole brain occurs without warning. The patient falls to the ground unconscious. Then, in the tonic phase, the patient goes rigid and often gives a short cry, due to the contrac tion of the diaphragm and chest muscles. The eyes may roll up or turn to one side, and the tongue may be bitten. After this, a period of jerky, clonic, spasms alternately flex and extend the muscles of the head, face, and extremities. During this phase, the patient may injure him- or herself as well as be incontinent. Cyanosis is generally marked. Breathing is deep, and there is sweating and salivation. Subsequent to the seizure, the patient may wake in a confused state (postictal twilight state) and even display some bizarre behavior. Sometimes patients are hard to arouse, sleep for hours, and awaken with headache or sore muscles. Although most tonic-clonic seizures last for only a few minutes, some patients develop a series of seizures with no letup, or a continuous prolonged seizure. This is a serious condition known as status epilepticus, which may lead to death if immediate care is not provided.

A variety of other generalized seizures have also been recognized. Sometimes patients exhibit only the tonic or clonic aspects of the seizure. Between the ages of 4 and 12 years, absence seizures often occur. These have been known as petit mal because they are of such brief duration, no more than a few seconds, that they often go unrecognized and untreated. During the brief lapse of consciousness, the child stares vacantly and neither speaks nor hears. Subsequently, activity is resumed with no period of stupor. Equally brief are atonic seizures, during which the child simply falls to the ground; myoclonic seizures, which are sudden, brief, and massive, involving either the entire body or confined to the extremities, face, or trunk; and infantile spasms, during which the child is jerked into a fetal position with the knees drawn up. Many children with infantile spasms are also mentally retarded.

Partial Seizures

All partial seizures begin in one part of the brain, and, because different parts of the brain control different parts of the body as well as mental and sensory functions, their signs and symptoms are varied and, often, quite complex. Many patients exhibit behaviors easily mistaken as psychiatric problems which can make accurate diagnosis difficult. It is also among victims of partial seizures that one is most likely to observe displays of bizarre, learned, culturally conditioned behavior.

Simple partial seizures have been variously called focal, focal motor, or focal sensory seizures. Although the symptoms may be motor, autonomic, psychic, sensory, or a combination, they are all linked to the affected area of the brain. The patient does not lose consciousness as a general rule, and the attacks last no more than 30 seconds. One type of simple partial seizure has been called the Jacksonian. It characteristically begins with the twitching of one foot or hand, and the patient retains consciousness. Until very recently, seizures were classed as Jacksonian even if the activity subsequently spread to both sides of the body and involved loss of consciousness. Today, however, such seizures are classed as partial but secondarily generalized.

Complex partial seizures are characterized by complex symptoms and, unlike simple partial seizures, by impairment of consciousness. Often the patient appears to be conscious but later has no recollection of the episode. These seizures are usually associated with the temporal or frontal lobe and often begin with an aura that warns of the impending attack. Auras may include any of a large variety of sensations. Some of those most commonly reported are nausea; faintness; dizziness; numbness of the hands, lips, and tongue; choking sensations; and chest pain. Less often, patients have reported visions, palpitation, or disturbances of smell or hearing. Some patients have sensations that may begin hours or even days before the seizure. These symptoms are called the prodrome and most often involve irritability or feelings of uneasiness. When psychomotor symptoms appear during a seizure, they are generally semipurposeful and inappropriate actions such as clumsy attempts to disrobe. Patients often stagger about uttering guttural sounds. Such behavior is often confused with psychiatric disorder and is often alarming to those present.

Secondarily generalized partial seizures occur when seizures with a focal onset spread throughout the brain and produce generalized tonic-clonic seizures. Because the generalized phase is so dramatic, patients and their families often overlook the focal onset. The presence of an aura indicates the presence of the focal onset and the need to observe the initial phase more closely.

The diagnosis of epilepsy and the classification of the type of seizure depend primarily on information obtained from the medical history. The first task is to determine whether the patient has epilepsy or has experienced another kind of brief, reversible alteration of consciousness or behavior. Subsequently, specifying the type of epileptic seizure is important for confirming the diagnosis and as a major guide in choosing the initial antiepileptic medication. Because the physician is most often unable to observe the patient or obtain an EEG reading during a seizure, an accurate medical history is of crucial impor tance. EEGs administered between seizures may or may not reveal patterns suggestive of epilepsy. Nevertheless, because they often do reveal abnormal discharges, routine administration of EEGs is of significant value in the evaluation of any patient with a history suggestive of epilepsy.

By conservative estimates, some 50 percent of patients can have their recurrent seizures controlled without side effects when optimal medical treatment is available. Another 30 percent can achieve seizure control but experience some side effects of the medication. For some patients whose seizures cannot be controlled by medication, surgery may be an option if a distinct piece of brain tissue that is causing the seizures can be identified, and if its removal will not cause unacceptable neurological deficits such as speech difficulty or memory loss.

History Antiquity

The antiquity of epilepsy is attested to by an ancient Akkadian text that speaks of a person whose neck turns left, whose hands and feet are tense and eyes wide open, from whose mouth froth flowed, and who lost consciousness. The Greeks referred to it as "the sacred disease" as well as "epilepsy," which means seizure and which may derive from the idea that all diseases represented attacks by supernatural beings. The term "sacred disease" is found first in the writings of Heraclitus and Herodotus, but its identification with epilepsy is made explicit in the book On the Sacred Disease, part of the Hippocratic collection of medical writings from about the year 400 B.C. and the first monograph on epilepsy we possess.

Underlying the great variety of explanations offered by the ancients lies the basic belief that epilepsy is an affliction or possession by a higher power and that its cure must be supernatural. The Romans called epilepsy morbus comitalis because the attack spoiled the day of the comitia, the assembly of the people. There was also the idea that the disease was contagious: The epileptic was unclean and whoever touched him or her might become prey to the demon. The idea that epilepsy was contagious was one of the factors that made the epileptic's life miserable and gave him or her a social stigma. To the ancients the epileptic was an object of horror and disgust and not a saint or prophet as has sometimes been contended. Wherever the physicians of antiquity wrote of the sacred disease, they meant epilepsy and differentiated it from hysterical attacks as well as from madness.

In the struggle between supernatural and scientific explanations of disease, science has gradually emerged victorious in the Western world. The fight, however, has been long and eventful, and in it epilepsy held one of the key positions. Showing both physical and psychic symptoms, epilepsy more than any other disease was open to interpretation both as a physiological process and as the effect of supernatural influences. The first record we have of the battle is in On the Sacred Disease, an attack on popular superstition that called epilepsy the "sacred" disease. It maintained that epilepsy was hereditary, that its cause lay in the brain, and its treatment was to be by diet and drugs as long as it had not yet become chronic. It is here we first find the fundamental statement that the seat of the disease is in the brain and that the brain is the organ of all psychic processes both normal and pathological. Moreover, according to the author of this work, not only epilepsy but all mental diseases were to be explained by disturbances in the brain.

Middle Ages Through the Eighteenth Century

During the Middle Ages, the literature on epilepsy propounded two contrasting views. On the one hand, the "falling evil" was bound to demoniac beliefs and theological speculations; on the other, physicians clung to the idea of a definite natural disease. Little effort was made to force the issue, however; physicians rarely discussed the theological aspects and seem, moreover, to have been unable to rid themselves of traditional definitions and explanations. By the end of the sixteenth century, this appears to have changed, the debate became open, involving the role of the devil, witchcraft, and various types of magical treatment. Despite many efforts to define epilepsy and classify types of seizures, little progress was made medically, although, gradually, the idea that epilepsy was a natural disease did gain more credence, especially after the Age of Enlightenment.

Nineteenth Century

By the beginning of the nineteenth century, epileptics were hospitalized, but unlike the insane, were allowed to go to mass on Sundays. Confined epileptics, however, became the object of systematic medical attention only in the early nineteenth century. The care of epileptics, especially children, progressed slowly. Only in 1838 were epileptic children in Paris transferred from the Hospital of the Incurably 111 to the Bicetre, where some kind of education was provided for them. The separation of hospitalized epileptics from the insane was motivated less from solicitude for the epileptics who might suffer from contact with the insane than from the belief that epilepsy was an infectious disease that would affect the insane even more than it did the healthy. The confinement of epileptics in separate wards of lunatic asylums became established procedure in Europe around 1850 and was soon followed by requests for special institutions for epileptics.

During the early part of the nineteenth century, the most valuable contributions to the medical history of epilepsy were made by physicians associated with hospitals and lunatic asylums, and it was then that new terminology, increased use of statistics, and interest in the psychiatric side of epilepsy developed. The terms grand and petit mal, absence, status epilepticus, and aura, for example, were in common usage and survive to this day. The growing use of statistics fostered investigations into the inheritabil-ity of epilepsy and determination of the various causes of the illness, prominent among which were fright, sorrow, and masturbation. Despite the increased attention paid to epilepsy, however, modern medicine's understanding is usually said to have begun around 1880, when the impact of John Hughlings Jackson's work in England and that of Jean Charcot in France began to be felt. Jackson outlined a neurological theory of epilepsy, while Charcot separated epilepsy from hysteria more emphatically than any of his predecessors. Jackson's principles were publicly demonstrated in 1888, by William Macewan, who was "probably the first surgeon to localize the cerebral focus by inference from the motor or sensory signs of the epileptic seizure" (Temkin 1971).

Jerrold E. Levy

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