Status Epilepticus

Disease Overview

Status epilepticus (SE) is considered a neurological emergency. Left untreated (or undertreated), prolonged seizures can cause permanent neurological injury or death. Rapid treatment must be initiated. If initial agents fail, it may be necessary to induce an iatrogenic coma. In any case, the person in status epilepticus must be closely watched, and often requires continuous EEG in order to confirm that the seizures have stopped not only clinically but electrically as well.

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Synonyms

• Convulsions
• Seizures

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Subdivisions

• Abdominal Epilepsy
• Akinetic Seizure
• Epilepsia Procursiva
• Febrile Seizures
• Focal Epilepsy
• Grand Mal Epilepsy
• Jacksonian Epilepsy
• Lundborg-Unverricht Disease
• Major Epilepsy
• Minor Epilepsy
• Myoclonic Astatic Petit Mal Epilepsy
• Myoclonic Progressice Familial Epilepsy
• Myoclonic Seizure
• Petit Mal Epilepsy
• Petit Mal Variant
• Psychomotor Convulsion
• Psychomotor Epilepsy
• Psychomotor Equivalent Epilepsy
• Pyknoepilepsy
• Status Epilepticus
• Temporal Lobe Epilepsy
• Unverricht-Lundborg-Laf Disease
• Unverricht Syndrome

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Signs & Symptoms

Epilepsy is a group of disorders characterized by electrical discharges in the brain. There are no established factors that bring on an epileptic seizure that are common to all patients. However, visual phenomena such as flickering lights or sunbursts, are frequently cited by people with epilepsy as preceding an attack. In certain patients the likelihood of having a seizure increases with stress, fatigue, insufficient food intake and/or the failure to take prescribed medications.

The Status Epilepticus are classified as follows:

Generalized Convulsive Status Epilepticus (GCSE): Overt (70 percent of GCSE): This consists of continuous tonic and/or clonic activity with impairment or loss of awareness.

Subtle (30 percent of GCSE): This is less obvious than the overt type, consisting of facial twitching, nystagmoid eye movements, or subtle jerking of the extremities.

Non-Convulsive Status Epilepticus (NCSE): Complex Partial Status Epilepticus: This type occurs in patients with a history of partial seizures, but can also arise as a result of acute injury (like a new stroke). This type may cause focal motor manifestations (like nystagmus), but can also cause more subtle findings such as confusion, personality changes or psychosis. Absence (Typical) Status Epilepticus: This occurs in patients with idiopathic generalized epilepsy. As with complex partial status, the clinical symptoms can be subtle, consisting of confusion or personality changes.

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Causes

The exact cause of epilepsy is unknown. Hereditary factors have been suggested as a possible cause in essential epilepsy. Some types of epilepsy occur as a symptom of other disorders, while others are thought to be caused by head injuries. In some families there may be a genetic predisposition to epilepsy, but scientists do not understand the hereditary factors that may make a person vulnerable to getting seizures.

The most common causes of recurring seizures in infants include: genetic inborn errors of metabolism; other metabolic disorders; developmental brain defects; injuries occurring a few months before birth or a few weeks after birth (perinatal); and/or a severe lack of oxygen (hypoxia). In children the typical causes of new-onset epileptic seizures can include: inflammation of the membranes that surround the brain and the spinal cord (meningitis); inflammation of the brain (encephalitis); brain abscesses and/or tumors; exposure to poisons or toxins; diseases that affect the blood vessel system (vascular diseases); degenerative diseases of the brain; and/or head trauma.

Epileptic seizures that occur in infants or children as a result of an abnormally high fever (febrile seizures) generally do not recur. In adults the beginning of epileptic seizures can sometimes be associated with a brain tumor, trauma to the head, stroke, cerebrovascular disease and/or degenerative brain disease. However, in many cases the cause cannot be identified.

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Affected populations

It is estimated that 150,000 people develop epilepsy each year in the United States. Per the MMWR report from the CDC, the overall prevalence for Epilepsy is 4.6/1000 in the general population while it is 4.1/1000 for population less than 15 years in age. The incidence of Epilepsy is more predominant in the extremes of age i.e. more frequent among children and older adults. Approximately 15 percent of people who have epilepsy have Status Epilepticus. Combined together approximately two to three million Americans have epilepsy but the majority of affected individuals are seizure free due to effective medications.

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Disorders with Similar Symptoms

Symptoms of the following disorders can be similar to those of Status Epilepticus. Comparisons may be useful for a differential diagnosis:

Wilson’s Disease is a rare inherited disorder that affects the liver, eyes and neuromuscular system. Symptoms develop due to the excessive accumulation of copper in body tissues, particularly the liver, brain and eyes. Early diagnosis and treatment of Wilson’s Disease may prevent serious long-term disabilities. Neuromuscular symptoms of Wilson’s Disease generally appear between the ages of 12 and 32 years. These symptoms may include drooling, joint pain (dysarthria), impaired speech (dysphasia), lack of muscle coordination, tremors, involuntary jerky muscle movements, muscle rigidity and double vision. Other late symptoms of Wilson’s Disease may include a decrease in cognitive abilities, behavioral changes, kidney stones, depression and other psychiatric disturbances. (For more information on this disorder, choose “Wilson’s” as your search term in the Rare Disease Database.)

Kok Disease (Hyperexplexia) is a very rare inherited disorder of the neurological system. People with Kok Disease have an excessive startle reaction to sudden and/or unexpected noise, movement or touch. When the individual with Kok Disease is startled, the head may arch back and there may be jerking muscle movements (myoclonic jerks). When startled the individual may also fall to the ground in a rigid position. Some people with Kok Disease also experience seizures. (For more information on this disorder, choose “Kok Disease” or “Hyperexplexia” as your search term in the Rare Disease Database.)

Myoclonus is a neurological movement disorder in which a skeletal muscle undergoes sudden, involuntary contractions resulting in jerky movements. There are 3 types of Myoclonus: Intention, rhythmical, and arrhythmic. Intention myoclonus is characterized by episodes of involuntary muscle contractions that are triggered by voluntary movements, such as a purposeful action. In arrhythmic myoclonus, muscle jerks are arrhythmic and sudden. The muscle jerking may be confined to a single muscle or involve the all of the skeletal muscles on one or both sides of the body. The stimulus for the onset of an episode may be sensory (visual, auditory, and/or tactile), or it may be fatigue, stress or anxiety. An extreme startle response may also be present. Rhythmical (segmental) myoclonus is characterized by very rapid and frequent muscle jerks. In contrast to Arrhythmic Myoclonus, this type of Myoclonus is not relieved by sleep and is not triggered by sudden stimuli or voluntary movements. Myoclonic muscle jerks may sometimes be confused with the muscle jerks and rigidity that occur in some forms of Epilepsy. (For more information on this disorder, choose “Myoclonus” as your search term in the Rare Disease Database.)

Narcolepsy is a rare neurological sleep disorder characterized by extreme unnatural drowsiness during the day, sudden loss of voluntary muscle tone (cataplexy), hallucinations, sleep paralysis, and/or disrupted sleep during the night. Symptoms usually begin between the ages of 10 and 20 years. The development and severity of symptoms vary greatly among patients. Exaggerated daytime drowsiness is usually the first symptom. The person with Narcolepsy may describe a feeling of sleepiness, tiredness, lack of energy, a “sleep attack”, and/or the inability to resist sleep. People with Narcolepsy who have cataplexy can fall asleep so suddenly that they appear to drop to the floor unconscious. In sleep paralysis, the person with Narcolepsy want to move but cannot do so. (For more information on this disorder, choose “Narcolepsy” as your search term on the Rare Disease Database.)

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Standard Therapies

SE is treated with anticonvulsant drugs that attempt to prevent and control seizures. The drugs that are currently used include phenytoin, valproic acid, carbamazepine, phenobarbital, clonazepam, ethosuximide (Zarontin), primidone, acteazolamide, paraldehyde, trimethadione, corticotropin and corticosteroids. Brain surgery for epilepsy that is caused by a brain tumor or drug-resistant temporal lobe epilepsy may be tried after medications have failed to stop seizures. Surgery is generally not performed until other treatment methods have failed. The success rate for such surgeries is approximately 55 to 70 percent.

It is very important to protect the epilepsy patient from self-injury during a seizure. Protective measures should include clearing the area of any object that is hard or sharp, loosening tight clothing and placing a flat, soft object under the head. The patient should be turned on the side and if possible something soft and flat (such as a pad or wallet) may be placed between the teeth. Restraint is not advised. The administration of artificial respiration should be attempted only if breathing does not start after the seizure has stopped. When the seizure is over, the patient should be allowed to sleep or be helped home if he or she seems confused. If the patient wants to sleep, the head and shoulders should be raised.

It is possible that some people with epilepsy who have had no seizures during an extended period of time (several years) may reduce or discontinue anticonvulsant medications under close supervision by a doctor.

The Food and Drug Administration (FDA) has approved the anti-convulsant drug Felbamate for use in hard-to-treat epilepsy such as Lennox-Gastaut. One of the main advantages of this drug is that it is not a sedative, so it will not make the user feel sleepy or sluggish. Felbamate is manufactured by Wallace Laboratories of Cranbury Run, NJ, and will be marketed under the brand name Felbatol. Due to occurrences of rare but serious side effects from this drug, physicians should contact the manufacturer or FDA before prescribing.

The Food and Drug Administration has approved the drug Lamotrigine (Lamictal) for the treatment of hard-to-control partial seizures. The drug is manufactured by Burroughs-Wellcome Company.

The drug fosphenytoin sodium (cerebyx) has been approved by the FDA for the treatment of epilepsy. Fosphenytoin sodium is manufactured by Warner-Lambert.

The drug tipiramate (Topamax) has been approved by the FDA for the treatment of epilepsy in addition to other medications. Topiramate is manufactured by Ortho-McNeil. In addition, Neurontin (gabapentin) has also been approved for use in the treatment of epilepsy.

The drug Diastat (diazepam suppository rectal gel) has been approved by the FDA for the treatment of epilepsy. Diastat is manufactured by Athena Neuosciences Inc.

The drug carbamazepine extended release capsules (Carbatrol) has been approved by the FDA for the treatment of epilepsy. Carbatrol is manufactured by Shire Pharmaceuticals.

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Clinical Trials and Studies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com.

Studies are underway on many experimental anti-convulsant drugs for the treatment of epilepsy. The website, www.clinicaltrials.gov, lists more than 100 clinical trials that deal with various investigational aspects of epilepsy. For additional information on treatment research, visit the websites of the organizations listed as resources in this report.

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References

TEXTBOOKS

Bennett JC, Plum F., eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996:2113-25

Adams, RD, et al., eds. Principles of Neurology. 6th ed. New York, NY: McGraw-Hill, Companies; 1997:313-41.

JOURNAL ARTICLES

Devinsky O, et al. A double-blind placebo-controlled study of remacemide hydrochloride in patients with refractory epilepsy following pre-surgical assessment. Seizure. 2002;11:371.

Kwan P, et al. Early identification of refractory epilepsy. New Engl J Med. 2000;342:314-19

Lowenstein D, et al. Status epilepticus. New Engl J Med. 1998;338:970-7.

Dreifuss FE, et al. A comparison of rectal diazepam gel and placebo for acute repetitive seizures. New Engl J Med. 1998;338:1869-75.

Vining EPG, et al. Gaining a perspective on childhood seizures. New Engl J Med. 1998;338:1916-18.

Sander R, et al. Refined mapping of the epilepsy susceptibility locus EJM1 on chromosome 6. Neurology. 1997;49:842-47.

Carpay HA, et al. Disability due to restrictions in childhood epilepsy. Developmental Medicine and Child Neurology. 1997;39:521-26.

Dichter MA, et al. New antiepileptic drugs. New Engl J Med. 1996;334:1583-90.

Yager J, et al. Sublingual lorazapam in childhood serial seizures. Am J Dis Child. 1988;142:931-32.

Herzog A, et al. Seizure control with clomiphene therapy. A case report. Arch Neurol. 1988;45:209-10.

Brandt L, et al. Control of epilepsy partialis continuans with intravenous nimodipine. Report of two cases. J. Neurosurg. 1988;69:949-50.

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Programs & Resources

• Assistance Programs
• Patient Organizations

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