Status Epilepticus

Status Epilepticus

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Status Epilepticus is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Convulsions
  • Seizures

Disorder Subdivisions

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

General Discussion

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.

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.

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.

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.

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.

Investigational Therapies

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.

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.

Resources

March of Dimes Birth Defects Foundation

1275 Mamaroneck Avenue

White Plains, NY 10605

Tel: (914)997-4488

Fax: (914)997-4763

Tel: (888)663-4637

Email: Askus@marchofdimes.com

Internet: http://www.marchofdimes.com



Epilepsy Foundation

8301 Professional Place

Landover, MD 20785-7223

Tel: (866)330-2718

Fax: (877)687-4878

Tel: (800)332-1000

TDD: (800)332-2070

Email: ContactUs@efa.org

Internet: http://www.epilepsyfoundation.org



PHP - Parents Helping Parents, Inc.

Sobrato Center For Nonprofits San Jose

1400 Parkmoor Avenue,

Suite 100

Santa Clara, CA 95126

USA

Tel: (408)727-5775

Fax: (408)286-1116

Tel: (866)747-4040

Email: info@php.com

Internet: http://www.php.com



NIH/National Institute of Neurological Disorders and Stroke

P.O. Box 5801

Bethesda, MD 20824

Tel: (301)496-5751

Fax: (301)402-2186

Tel: (800)352-9424

TDD: (301)468-5981

Internet: http://www.ninds.nih.gov/



AED Pregnancy Registry - Massachusetts General Hospital

121 Innerbelt Road, Room 220

Somerville, MA 02143

Tel: (617)724-9550

Fax: (617)724-8307

Tel: (888)233-2334

Email: holmes.lewis@mgh.harvard.edu

Internet: http://www.aedpregnancyregistry.org



American Epilepsy Society

342 North Main Street

West Hartford, CT 06117-2507

Tel: (860)586-7505

Fax: (860)586-7550

Email: khucks@aesnet.org

Internet: http://www.aesnet.org



Epilepsy Canada

2900 John St., Suite 402

Markham

Ontario, L3R 5G3

Canada

Fax: 9055139461

Tel: 8777340873

Internet: http://www.epilepsy.ca



Organisation For Anti-Convulsant Syndrome

131 Summertrees Road

Ellesmere Port

Cheshire, CH66 2BE

United Kingdom

Tel: 01513565949

Email: enquiries.oacs@gmail.com

Internet: http://www.oacs-uk.co.uk/



New Horizons Un-Limited, Inc.

811 East Wisconsin Ave

P.O. Box 510034

Milwaukee, WI 53203

USA

Tel: (414)299-0124

Fax: (414)347-1977

Email: horizons@new-horizons.org

Internet: http://www.new-horizons.org



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Dravet.org

P.O. Box 797

Deale, MD 20751

USA

Tel: (443)607-8267

Email: info@Dravet.org

Internet: http://www.dravet.org



LGS Foundation, Inc.

192 Lexington Avenue

New York, NY 10016

Tel: (718)374-3800

Email: christina@lgsfoundation.org

Internet: http://www.lgsfoundation.org



Matthew's Friends

St. Piers Lane

Lingfield

Surrey, RH7 6PW

United Kingdom

Tel: 01342836571

Fax: 07884054811

Tel: 07884054811

Email: enq@matthewsfriends.org

Internet: http://www.matthewsfriends.org



Child Neurology Foundation

2000 West 98th Street

Bloomington, MN 55431

USA

Tel: (952)641-6100

Fax: (952)881-6276

Tel: (877)263-5430

Email: jstone@childneurologyfoundation.org

Internet: http://www.childneurologyfoundation.org



CURE: Citizens United for Research in Epilepsy

223 W. Erie

Suite 2SW

Chicago, IL 60654

Tel: (312)765-7118

Fax: (312)255-1801

Tel: (800)765-7118

Email: info@CUREepilepsy.org

Internet: http://www.CUREepilepsy.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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