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Dr. Alim Akhtar Bhuiyan Consultant Neurologist and Epilepsy Specialist Apollo Hospitals ,Dhaka
Overview of Epilepsy & Seizures Epilepsy is a recurrent seizure disorder caused by abnormal electrical discharges from brain cells, often in the cerebral cortex. It is not a distinct disease, it is a group of disorders for which recurrent seizures are the main symptom. Different forms of epilepsy are either secondary to a particular brain abnormality or neurological disorder, or are said to be "idiopathic," without any clear cause. Normally, nerve transmission in the brain occurs in an orderly way, allowing a smooth flow of electrical activity. A seizure occurs when these neurons generate unco-ordinated electrical discharges that spread throughout the brain. This can occur with both normal and abnormal nerve cells. Epilepsy Incidence Seizure disorders are a common neurological problem. In the United States alone, it has been estimated that more than 4 million people have some form of epilepsy.. The prevalence of epilepsy (defined as the total of the population suffering from a disorder at a particular time) has been estimated to be about 5 to 8 in every 1000 people, The number of patients with Epilepsy is more common in developing countries including Bangladesh .
(including a special deep area called the hippocampus, which is involved in memory). The reason for this likelihood may be that nerve cells in these areas are particularly sensitive to certain situations that can provoke abnormal electrical transmission. Examples include sensitivity to decreased oxygen levels, metabolic changes, and infection, any of which may lead to a seizure. Many types of brain abnormalities can be responsible for producing seizure activity. Abnormal discharges may spread to other cells in a local area or to remote areas of the brain, resulting in intermittent disturbance in the brain's normal functions. Changes in brain biochemistry and communication between brain cells occur. These basic neurofunctional abnormalities that lead to epilepsy produce the clinical symptoms that are seen. In turn, recurrent seizures or prolonged seizures can cause injury to the brain. Seizures that last longer than 20 to 30 minutes can damage the brain's neurons. A seizure is often divided into different parts. The aura is a period or warning prior to a seizure. Patients may experience unusual smells, visual symptoms, or feelings. The seizure itself is known as the ictus. The period of time after the seizure is called the postictal state. Seizures were depicted by prehistoric man in cave paintings. Hippocrates wrote of epilepsy and of its relationship to the brain. Epilepsy also is described in the Bible. Writings from 4000 years ago depict epileptics as possessed by demons. Julius Caesar, the great Russian novelist Dostoyevsky, and King Charles II all are said to have had seizures.
Types of Seizures
Seizure Types There have been many attempts to categorize seizures, based on both the causes of seizures as well as the different seizure subtypes. A well-recognized classification system is the International Classification of Epileptic Seizure. This divides seizure types by the location in the brain that they originate from. The two main categories of seizures include partial seizures and generalized seizures Partial seizures are those that begin in a focal or discreet area of the brain. This type can be further subdivided into:
Simple partial: No change in consciousness occurs. Patients may experience weakness, numbness, and unusual smells or tastes. Twitching of the muscles or limbs, turning the head to the side, paralysis, visual changes, or vertigo may occur. When motor symptoms spread slowly from one part of the body to another, this "epileptic march" has been termed jacksonian epilepsy (first described by Hughlings Jackson). Complex partial seizures (temporal lobe): Consciousness is altered during the event. Patients may have some symptoms similar to those in simple partial seizures but have some change in their ability to interact with the environment. Patients may exhibit automatisms (automatic repetitive behavior) such as walking in a circle, sitting and standing, or smacking their lips together. Often accompanying these symptoms are the presence of unusual thoughts, such as the feeling of deja vu (having been someplace before), uncontrollable laughing, fear, visual hallucinations, and experiencing unusual unpleasant odors. These interesting symptoms are thought to be caused by abnormal discharges in the temporal lobe.
Generalized seizures involve larger areas of the brain, often both hemispheres (sides), from the onset. They are further divided into many subtypes. The more common include:
Tonic-clonic (grand mal): This subtype is what most people associate with seizures. Specific movements of the arms and legs and/or the face may occur with loss of consciousness. A yell or cry often precedes the loss of consciousness. Prior to this, patients may have an aura (an unusual feeling that often warns the patient that they are about to have a seizure). The person will abruptly fall and begin to have jerking movements of their body and head. Drooling, biting of the tongue, and
incontinence of urine may occur. When the jerking movements stop, the patient may remain unconscious for a period of time. The seizure usually lasts 5 to 20 minutes. They often awaken confused and may sleep for a period of time. The patients may experience prolonged weakness after the event; this is termed Todds paralysis.
Absence (petit mal): Loss of consciousness only occurs, without associated motor symptoms. Usually there is no aura, or warning. The loss of consciousness is brief; the patient may appear to be involved with the environment and briefly stop what they are doing, stare for 5 to 10 seconds, and then continue their activity. No memory of the event exits. Subtle motor movements may accompany the alteration in consciousness. Myoclonic: Myoclonic seizures are characterized by a brief jerking movement that arises from the central nervous system, usually involving both sides of the body. The movement may be very subtle or very dramatic. There are many different syndromes associated with myoclonic seizures, including juvenile myoclonic epilepsy, West syndrome and Lennox-Gastaut syndrome. Most cases of myoclonic epilepsy occur during the first 5 years of life.
West Syndrome West syndrome involves a group of symptoms including infantile spasms, retardation of psychomotor development, and a particular abnormality on the electroencephalogram (EEG) known as hypsarrhythmia. Infantile spasms are characterized by a particular posturing of the infant's body, in which the child assumes a jack-knife, or folded, position. These spasms may occur frequently in the course of the day or may be continuous. Neurological problems are ultimately found in most of these children. The hypsarrhythmia pattern seen on the EEG is a grossly disorganized pattern of electrical brain activity. It is often difficult to control the seizures in this syndrome because they usually respond poorly to most anticonvulsant medications. Lennox-Gastaut Syndrome Lennox-Gastaut syndrome is characterized by the early onset of a common seizure type called minor motor seizures. These seizures include the aforementioned myoclonic seizures, atypical absence seizures, and atonic seizures. Atypical absence seizures may involve staring and brief episodes of unconsciousness. They may occur in cycles and are associated with EEG findings different from those seen in typical absence seizures. Atonic seizures may be associated with sudden loss of muscle tone. Status Epilepticus Status epilepticus is prolonged, repetitive seizure activity that lasts more than 20 to 30 minutes, during time which the patient is unconscious. Status epilepticus is a medical emergency with a significantly poor outcome; it can result in death if not treated aggressively. Its causes include improper use of certain medications, stroke, infection, trauma, cardiac arrest, drug overdose, and brain tumor.
Genetic factors: It is now accepted that some persons may have a genetic predisposition to the development of seizures. There is also an increased incidence of epilepsy in relatives of those with a seizure disorder. Head injury: Seizures may develop at or around the time of injury or years after (usually not more than two years later). They may occur with either an "open" or "closed" head injury. Stroke/cerebrovascular disorders: Seizures can occur at the time of a stroke or many years later. They may occur with strokes that result in lack of blood flow to the brain or with those that involve bleeding into or around the brain. Metabolic disturbances: This group of disorders changes levels of various metabolic substances in the body. These disease states sometimes result in seizures.
Electrolyte disturbances (altered levels of sodium, calcium, or magnesium) Hypoglycemia (low blood sugar) or hyperglycemia (elevated blood sugar) Renal failure (kidney disease) with uremia (increased urea in the blood) or changes that occur around the time of kidney dialysis Hepatic failure (severe liver disease) and elevation of associated toxins Hypoxia (lowered oxygen delivery to the brain)
Toxic causes: The presence of certain drugs can cause seizure activity. In addition, abrupt withdrawal of some substances can lead to seizure activity. These substances that may induce seizures include the tricyclic antidepressants, lithium, antipsychotic medications, aminophylline, and high doses of penicillin. Illicit drug use, particularly cocaine, heroine, amphetamines, and PCP, can cause seizures. Alcohol withdrawal can be associated with seizure activity. These seizures usually occur 12-24 hours after the last drink but can occur up until 48 hours or more after binge drinking. Withdrawal from prescription drugs and agents such as barbiturates and narcotics can result in seizure activity. Infections: Infections of the nervous system may result in a lowered seizure threshold. These may include meningitis (infections of the coverings of the brain and spinal fluid), encephalitis (infection of the brain itself), and HIV (human immunodeficiency virus), and related infections. Tumors and space-occupying lesions: Brain tumors, both malignant (cancerous) and benign, may be associated with seizures. The anatomic location of the abnormality influences the likelihood of having seizures. Degenerative disorders: There are many neurodegenerative disorders that are accompanied by seizures. These include tuberous sclerosis, neurofibromatosis, Tay-Sachs disease, phenylketonuria (PKU), and Sturge-Weber syndrome. Brain damage in infancy: Cerebral palsy secondary to lack of oxygen, infection, or trauma is associated with epilepsy. Febrile seizures: These are an age-associated form of epilepsy that may present as a single seizure or may be recurring. They are associated with a high fever in children 3 months to 4 years of age and occur in 3% 4% of children.
Disorders That Mimic Seizure Disorders True seizure disorders must be differentiated from a variety of problems whose symptoms approximate or closely resemble those of epilepsy. These include other conditions include cerebrovascular (stroke-related) disorders, migraine, narcolepsy, syncope (fainting), and anxiety and other psychiatric disorders. Another type of spell well known to physicians is the so-called pseudoseizure, or more properly nonepileptogenic seizure. These spells are not triggered by nerve cell discharges that cause true epilepsy, although the patient may experience muscle twitching and even apparent loss of consciousness. These spells have a psychiatric component and often coexist in persons who have true epilepsy. EEG monitoring can help distinguish disorders that mimic epilepsy from true seizures.
also is helpful to distinguish seizure subtypes, partial or generalized; time of day of the event, including whether the seizure occurred during wakefulness or sleep; and any known triggers, such as a flickering light, severe sleep deprivation, or dehydration. Thorough general physical and detailed neurological examinations also should be performed. Diagnostic Testing for Epilepsy Laboratory data utilized in the diagnostic evaluation of patients with seizure disorders may include CAT scan imaging, magnetic resonance imaging (MRI), and electroencephalography (EEG). A complete blood laboratory panel, including drug-toxic screening, and urinalysis are usually performed. CAT scan brain imaging is often the first radiological study obtained, especially in the emergency room setting. It can indicate pathology such as a brain tumor, stroke, brain hemorrhage, acute or remote trauma, and infection. Limitations include inability to evaluate certain structures in the brain in detail, particularly the temporal lobes of the brain, often the first sites of seizure activity. MRI brain imaging is performed for many patients with seizure disorders. It has the ability to visualize in great detail much of our brain anatomy. Subtle asymmetries in relevant structures, as well as undetected mass lesions, small strokes, and evidence of trauma may be uncovered. The use of a contrast agent (injected into the vein) can enhance the ability to show underlying abnormalities. EEG (also known as "brain wave") provides information about the electrical activity in the brain. Unlike CAT or MRI, it does not produce an image of the brain, but supplies information about the function or dysfunction of parts of the brain. Multiple small electrodes are placed at specific points on the scalp on both sides of the head to record activity generated mainly by the cerebral cortex. Brain wave activity is usually recorded for 30-45 minutes. Many patients will also be tested while hyperventilating (breathing rapidly and deeply), when flashing lights are flashing, while sleeping, or when sleep deprived. The brain normally exhibits particular patterns on the EEG during wakefulness, drowsiness, and sleep. The duration and character of normal brain-wave activity can be used as a baseline for comparison with abnormal waves on an EEG.
Some of the findings on EEG are specific to particular disorders and subtypes of epilepsy. Activity during a seizure can be identified by a pattern on the graph indicating epilepsy called epileptiform. Correlating this type of data with clinical symptoms of seizures often helps make an accurate diagnosis. The EEG recording between seizures is often abnormal in patients with epilepsy, and so can be helpful in this setting. EEG can be combined with video monitoring to record activity both electrically and visually. EEG can also be performed in a long-term care setting with a portable ambulatory unit, to monitor patients for up to many days at a time. Other uses of the EEG include evaluation of altered states of consciousness, brain damage, metabolic-toxic injury, and suspected brain death.
There is still some controversy over whether to treat a patient who has had only a single seizure. Approximately 75% of seizure sufferers have only one seizure and no reoccurrence. In making treatment decisions, it is helpful to look at risk factors that may predict a second seizure. These include lesions of the brain, an abnormal EEG, or a family history of seizure disorders. Anticonvulsants can reduce the risk of further seizure activity. People who have had more than one seizure should probably be treated with anticonvulsants.
Valproate (Depakote) has been in use for more than 20 years. It can be prescribed for a broad spectrum of anticonvulsant needs, including partial seizures, generalized tonic-clonic (grand mal) seizures, absence (petit mal), and myoclonic epilepsy. Its mechanism of action is thought to be related to the effect of a brain substance known as GABA (gamma-aminobutyric acid). It is available in oral form and must be taken 2 to 3 times per day for adequate dosing. Drug levels must be monitored, as well as liver function, and blood count. The drug's suggested therapeutic window is 50100mg/L. Side effects include hepatotoxicity (liver damage), nausea, weight gain, allopecia (hair loss), and tremor.
Trileptal (oxcarbazepine) is indicated for monotherapy (used alone) in adults who have partial seizures and can be used in children as add-on therapy for partial seizures. The most common side effects include dizziness, sleepiness, nausea, and imbalance, but these do not warrant clinical observation. Zonegram (Zonisamide) is approved for use in adults as adjunctive therapy for partial seizures. It has however, been used fairly extensively in other countries for use in other seizure types including generalized seizures, myoclonic seizures and absence seizures. Side effects can include dizziness, imbalance and fatigue. Individuals who are allergic to sulfonamide drugs should not use Zonisamide since it is a derivative of this class of drug
what seizures are, and what symptoms may warn of their onset when to notify a physician about any change in symptoms details of medication dosing, side effects, and importance of medication compliance (taking medications exactly as they are directed and not missing doses) importance of laboratory monitoring tests with some drugs advice on living a healthy lifestyle and avoiding things that may trigger seizure activity (sleep deprivation, increased stress, alcohol and drug abuse) counseling women of childbearing age about epilepsy, medications, and pregnancy
Although the laws vary somewhat from state to state, most states have rules regarding when seizure patients can legally resume driving. Typically, patients must have been seizure-free 6 months to a year. While physicians generally are not required to report patient seizures to their state motor vehicle departments, patients are expected to comply with the laws in their state.