Clinical and Health Affairs
Caring for the Patient with Seizures: A 21st Century Approach
By Robert J. Gumnit, M.D.
Epilepsy is a condition that affects less than 2 percent of the population. In the past, people with recurring seizures often suffered their entire lives. Today, most seizure cases can be controlled with appropriate medication and follow-up. However, some patients require specialized care. This article reviews the current approach to diagnosing and treating epilepsy, which is based on the goals and standards set by the American Academy of Neurology.
What is taught to medical students often becomes obsolete as they practice. This is particularly true for conditions that cannot be diagnosed with a simple blood test or X-ray. One of those is seizure disorder.
One out of every 11 people will have a seizure sometime in their life.1 One-third of those people will have simple febrile seizures. Another third will have only one seizure. The other third will have seizures throughout their lives. In some, the seizures are secondary to problems such as heart block and other heart-rhythm disturbances, disturbances in circulation, and respiratory problems; in others, the seizures have a psychogenic origin. About 1.6% of the general population has been diagnosed with epilepsy, a term that means that a patient has had more than one unprovoked seizure on more than one occasion.2 Epileptic seizures arise from a disturbance of function of neuronal systems in the cerebral cortex, especially from the five-layered cortex of the mesial temporal lobe and the six-layered cortex of the frontal lobe.
The occurrence of any type of seizure is severely disruptive for the patient. The anxiety of not knowing if or when another one will strike is significant and often debilitating. Having uncontrolled seizures makes it difficult to obtain a driver’s license, buy health insurance, get a job, travel independently, or date and marry. It is no wonder that so many people in the 20th century who suffered from uncontrolled seizures were depressed and socially withdrawn.1 Today, however, diagnosis and treatment has advanced to the point where complete control of seizures without side effects is possible. Thus, physicians can now offer their patients with seizures hope that they previously could not. This article outlines the current approach to diagnosing and treating patients who have seizures.
Diagnosing Patients with Seizures
Medical care of patients with seizure disorder has advanced tremendously in the last three decades. For the majority of cases, care can be provided by a primary care physician. For a minority of patients, care is best delivered by a multidisciplinary team of epilepsy experts. The American Academy of Neurology (AAN) has issued detailed guidelines and standards for working with patients with seizure disorder.3 The following principles are based on those evidence-based guidelines.
1. Reach a precise diagnosis. It is important to find out whether the patient has experienced an epileptic seizure, a nonepileptic seizure, or something else. Although crucial to successful treatment, this may be difficult to determine. Much depends on the knowledge, skill, and patience of the physician obtaining the history, which often must be gleaned from family members and friends who may have witnessed what happened to the patient and are better able to describe it than the patient. Many patients do not remember what happened prior to and during the seizure.
Modern diagnostic techniques including long-term video EEG, specialized high-resolution MRI studies, and ictal SPECT recordings are usually only available at comprehensive epilepsy centers. These are expensive and time-consuming tests that require a high level of skill to perform. A patient whose condition is not well-controlled should be referred for such testing in order to achieve a more exact diagnosis. The information from these tests can help the epileptologist pinpoint the type of seizure, the type of epilepsy syndrome, and the location of the epileptic focus.
2. Identify patients with difficult problems and refer them early. Continued seizures are not good for the brain, and the disability that goes along with them is not good for the soul.
Kwan and Brodie have demonstrated that the patient’s response to the first medication given for seizures is a powerful predictor of their prognosis.4 Of those they studied in whom the first antiepileptic drug was not effective, only a small percentage ever became seizure-free with additional anticonvulsant treatment. After two appropriate antiepileptic drugs had been tried and the patient still suffered from seizures, the likelihood of that patient becoming seizure-free was almost nil. For this reason, a number of groups, including the National Association of Epilepsy Centers,5 the AAN,3 the Centers for Disease Control and Prevention,6 and groups in Britain and Singapore7-10 have recommended that patients who do not respond to therapy provided by a primary care physician after three months or by a neurologist within a year should be referred to a comprehensive epilepsy center. A working approach to caring for the patient with seizures who may need more complex care is outlined in the Figure.
It is important to note that the needs of the patient with intractable seizures can best be met when the relationships among the primary care physician, general neurologist, and epilepsy specialist are well-developed and cooperative. It is the responsibility of the primary care physician to recognize the disease and refer early; the epileptologist should develop a clear and specific treatment plan that he or she, along with the primary care physician or general neurologist, can together carry out.
Patients whose seizures are refractory or brittle require the care of a multidisciplinary team that not only provides an appropriate prescription of antiepileptic drugs and surgery but also looks into the patient’s genetic, cognitive, and psychological morbidities. Epilepsy specialists also can help women of child-bearing age avoid treatments that may cause birth defects. If possible, a woman of child-bearing potential with seizures should be referred to an epilepsy center for guidance prior to becoming pregnant, as soon as possible after becoming pregnant, or immediately after having a seizure.
3. Specify the seizure type and syndrome. In addition to distinguishing whether a patient is experiencing epileptic or nonepileptic seizures, physicians need to determine whether the patient is experiencing localization-related seizures or primary, generalized seizures.11 The recent revision of the ICD-9 classification of diseases provides a clear outline for classifying seizures.12 Appropriately classifying seizures serves as a good first cut in choosing therapy and therapeutic agents. Although resective surgery currently is not indicated for primary generalized epilepsy, it should be considered early (not as a treatment of last resort) for localization-related epilepsy. Similarly, while some medicines are appropriate and useful for localization-related epilepsy (eg, carbamazepine and the almost-outmoded phenytoin), they make primary generalized seizures worse. Diagnosing the epilepsy syndrome also is essential to the selection of both medical and surgical therapies.
Treating the Patient with Epilepsy
The treatments most appropriate for the seizure type and epilepsy syndrome should be initiated early on. In the case of a patient who is having epileptic seizures, the brain has to be treated primarily; if the seizures are nonepileptic events, the underlying disorder needs to be treated primarily.13 Physicians have several options for treating patients with epileptic seizure disorders.
■ Antiepileptic Drugs
Results of a recent survey published by the Centers for Disease Control and Prevention found that about 75% of the population taking medications for seizures did not report having a seizure in the past three months.2 This goes along with the rule of thumb that 80% of people can easily have their seizures controlled by medication. Primary care providers should learn how to use two or three powerful antiepileptic drugs for each major type of epilepsy. If two of those medicines fail to control a patient’s seizures, he or she should be referred to a neurologist or an epilepsy center.
A handful of patients with localization-related epilepsy have simple partial seizures that present either as sensory or psychic changes or focal motor seizures. These patients may be difficult to treat and may experience debilitating side effects. Often, these patients have complex partial seizures—seizures that are accompanied by a disturbance of consciousness. Some physicians still use antiquated terms such as “temporal lobe seizures” or “psychomotor seizures” to describe these phenomena. However, complex partial seizures do not always arise from the temporal lobe, although some of the most dramatic examples do have their origin in that part of the brain.
Primary care physicians can feel comfortable initiating treatment for complex partial seizures with carbamazepine (Tegretol, Carbatrol), lamotrigine (Lamictal), or levetiracetam (Keppra). All of these medications are now available in generic form. Each has its advantages and disadvantages. Lamotrigine (Lamictal) is particularly difficult to use in women who are pregnant, carbamazepine (Tegretol) may cause birth defects, and carbamazepine (Tegretol) can cause hyponatremia (but not as often as oxcarbazepine [Trileptal]).14 Of the three drugs, levetiracetam is the least likely to interact with other medications the patient may be taking, as it is excreted by the kidneys. In addition, levetiracetam causes less sedation, but it may cause headaches, irritability, and depression. It also is rather expensive.
Primary generalized seizures generally respond well to valproic acid (Depakote) and its cousin (Depakene). They also may respond well to lamotrigine. Valproic acid, in particular, is associated with birth defects, has a very short half-life, and causes nausea. One of the extended-release formulations is infinitely superior. Both of these medicines are available in a generic form.
■ Surgical Treatment
Surgery may benefit a small number of patients who cannot be treated adequately with medication. At present, surgical treatment can be divided into two broad categories: resective and implantation of stimulators.15 The outcomes for resective surgery have improved greatly in recent decades. As measured by seizure freedom for one year while still taking medications after resection, the rate of successful outcomes ranges from over 90% for patients with mesial temporal sclerosis and other lesions in the temporal lobe to better than 50% for patients who have nonlesional problems in other parts of the cerebral cortex. The mortality rate is almost zero percent, and morbidity, in the hands of an experienced surgical team, is less than 2%.
A number of devices may be surgically implanted. The vagus nerve stimulator, which has been approved for epilepsy, is expensive and is only slightly to moderately effective in the majority of patients.16 New deep-brain stimulation techniques and computerized recognition of seizure onset combined with cortical stimulation techniques are currently being studied and may be available soon. Thus far, the evidence indicates that they, too, are a fallback to be used for patients who have not responded to medications and who are not suitable candidates for resective surgery.
Surgery, when carried out on appropriately selected patients, has a very low morbidity rate and a mortality rate of practically zero percent. Recovery is usually quick, and patients are able to resume their daily activities in a few weeks.
The following criteria can help identify patients who may benefit from surgery:
The patient has failed at least two standard antiepileptic medications or suffers so much from side effects that he or she is unable to lead a productive life,
The patient’s seizure type has been accurately diagnosed and classified with the help of video EEG monitoring,
The patient’s epilepsy syndrome has been carefully identified, and Appropriate imaging studies have been carried out.
Computerized tomography is rarely helpful in evaluating patients with seizures for surgery unless the seizures are caused by a brain tumor or a vascular abnormality. Most patients require high-resolution MRI with thin cuts perpendicular to the axis of the temporal lobe and multiple parametric studies (including T1, T2, FLAIR sequences). At a minimum, a good 1.5-T MRI unit is needed to identify mesial temporal sclerosis. A more powerful unit may be needed to detect the presence of migration disorders and other developmental abnormalities. These developmental abnormalities comprise a large portion of the so-called idiopathic or cryptogenic cases of epilepsy. Ictal SPECT testing should be done in patients in whom the epileptic focus occurs in the lateral cortex of the temporal lobe or elsewhere in the brain. Positron emission tomography also may be useful.
Although these studies are expensive, they help identify patients who will benefit from surgery. The outcomes of surgery can be very gratifying, as more than 90% of patients with unilateral mesial temporal lobe sclerosis can be helped with surgery and the cure rate approaches 80%. Even the patient with no identifiable lesion whose seizures arise from the cortex of the brain has a better than 50% chance of substantial improvement in the hands of an experienced team.
Primary care physicians play a crucial role in caring for patients with episodic seizure disorder. They need to diagnose them as soon as possible and, once the correct diagnosis has been made, provide effective treatment and ongoing supervision, ideally working closely with a comprehensive epilepsy center or, if one is not available, a neurologist. Early referral to a neurologist or epilepsy specialist is important for patients with uncontrolled seizures.
The longer people have seizures, the harder they are to treat. The earlier the physician and patient understand the nature of the disorder, the better the outcome of therapy. If a patient has had what appears to be a seizure and does not respond to a major antiepileptic drug, then that patient should be referred to an epilepsy specialist immediately, as further drug treatment without an accurate diagnosis becomes a matter of chance. With the large array of modern treatments and the ready availability of epilepsy consultation in Minnesota, we have an opportunity to help our patients live seizure-free lives. MM
Robert J. Gumnit, M.D., is president MINCEP Epilepsy Care in Minneapolis and an adjunct professor of neurology and pharmacy at the University of Minnesota.
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