ddhealthinfo.org

EPILEPSY

Background
Medical Management Considerations
References
Resources for Families
Advisory Committee
Publication Information

Learning Points

  • Restate that epilepsy is estimated to occur in 1 in 100 to 1 in 200 in the general population and occurs in 1 in 400 individuals with mental retardation.
  • Recognize that in 70% of cases no etiology for the epilepsy can be identified.
  • Differentiate between partial seizures, generalized seizures, unclassified epileptic seizures and status epilepticus.
  • Delineate the medical management hierarchical approach to epilepsy (with less invasive treatment options to be employed first), to include:
    • Monotherapy
    • Polypharmacy
    • Ketogenic diet (age 2-12 years); effective in 25-30% of cases
    • Vagal nerve stimulation; effective in 20-50% of cases
    • Epilepsy surgery; effective in 80% of cases with careful selection criteria
  • Refer families to appropriate resources on epilepsy.

BACKGROUND
back to top

Description and Cause

Epilepsy is defined as a neurological disorder manifested by two or more unprovoked seizures. A seizure is a behavioral alteration due to abnormal and excessive electrical brain activity. A person who has multiple seizures due to fever, trauma, infection, etc. is not considered to be epileptic. Epilepsy may be caused by genetic conditions, head injury, stroke, brain tumor, toxic poisoning or severe infections like meningitis and encephalitis. These causes may occur during the prenatal, perinatal or postnatal period. In 70% of cases no etiology can be identified.

Occurrence

1:100 to 1:200 individuals in general population and 1:4 individuals with mental retardation suffer from epilepsy.

Characteristic Features

The International League Against Epilepsy has proposed a system of classification which includes partial seizures, generalized seizures, unclassified epileptic seizures and status epilepticus.

I. Partial Seizures

  • Originates in one discrete area of the brain, but may generalize
  • Typically last for a short time (two to five minutes)
  • Most common form of seizure among adults

Simple Partial (without impairment of consciousness)

  • Uncontrollable jerky motions of a body part
  • Sight, hearing or smelling alterations
  • Sudden sweating or flushing
  • Nausea
  • Feelings of fear, sadness, anger or happiness

Complex Partial (with impairment of consciousness)

  • Trance-like state lasting from a few moments to several minutes
  • Non-suppressible body movements
  • Automatisms (random, purposeless activity)
  • Motor seizures preceded by an aura (feelings of fear, abdominal discomfort, dizziness, strange odors and sensations, etc.)

II. Generalized Seizures

  • Partial seizures secondarily generalized
  • Primarily generalized seizures
  • Originates in the brain in a bilateral symmetrical fashion
  • Less common than partial seizures
  • Variable frequency
  • Many types (see below)

Typical Childhood Absence (petit mal) – Non-Convulsive Seizures

Note: The vast majority of “petit mal” in people with mental retardation is behavioral, but those due to seizure are more likely complex partial seizures rather than absence seizures.

  • Seizures between ages 3 and 8 years; usually diminish or disappear in adulthood
  • Sudden loss or impairment of consciousness (blackout) lasting 5 to 30 seconds
  • No aura preceding seizures
  • Upward staring of the eyes or eye blinking
  • Slight rhythmic movements of the facial muscles, or arms and legs
  • Usually no memory that seizures occurred
  • Seizures can be induced by hyperventilation
  • EEG: 3 Hz spike and wave complexes

Tonic-clonic (grand mal) – Convulsive Seizures

  • Onset of seizures at any age
  • Seizures lasting usually under 5 minutes
  • Occasionally signaled by involuntary scream
  • Initial stiffening of muscles (tonic phase), followed by a period of alternating relaxation and contraction (clonic phase)
  • Complete loss of consciousness
  • Arching of the back
  • Reddening of the face
  • Momentary cessation of breathing during tonic phase
  • Clenched hands and jaw
  • Eyes wide open; pupils fixed and dilated
  • Exhaustion and frequent sleeping following seizure
  • Tongue/cheek biting and loss of sphincter control
  • Subsequent confusion, nausea, sore muscles
  • No memory of seizures

Myoclonic

  • Sudden, brief, massive muscle jerks involving all or only some muscles
  • Repetitive muscle contractions
  • Seizures may occur in clusters
  • Can be seen in acquired & familial disorders
  • Prognosis and response to treatment varies according to etiology

Atonic (Astatic)

  • Seizures begin between ages 2 and 5 and are uncommon in adults
  • Seizures lasting 10 to 60 seconds
  • Sudden, brief loss of muscle tone
  • Loss of consciousness
  • May fall to ground if standing, or may be fragmentary and lead to head drop
  • Quick recovery after seizures
  • Frequently associated with myoclonic jerks

III. Status Epilepticus

Status epilepticus is most commonly caused by anticonvulsant drug withdrawal. It is estimated that 8% of people with generalized seizures experience status epilepticus. (Return to the index for a full document on status epilepticus.)

Definition

  • Successive seizures with no intervening periods of consciousness or seizures lasting twenty minutes or more
  • Convulsive or non-convulsive seizures

Common Causes

  • Central Nervous System infections
  • Tumors
  • Anoxia
  • Congenital brain malformations
  • Metabolic disorders
  • Drugs
  • Neurofibromatosis
  • Tuberous sclerosis
  • Mental retardation or learning disabilities

Status epilepticus is more common at the extremes of life and is associated with high morbidity and mortality

MEDICAL MANAGEMENT CONSIDERATIONS
back to top

Note: These considerations are in addition to the normal medical care provided to an individual without epilepsy.

Management of epilepsy is usually predicated upon the assumption that the least invasive treatment options should be employed first, which generally suggests the following hierarchical approach:

  • Monotherapy
  • Polypharmacy (see below)
  • Ketogenic diet (age 2-12 years); effective in 25-30% of cases
  • Vagal nerve stimulation; effective in 20-50% of cases
  • Epilepsy surgery; effective in 80% of cases with careful selection criteria

Note: There is some debate over the optimal age range for the last three approaches.

Uncomplicated and well-controlled epilepsy can easily be managed by the primary care physician, particularly when monotherapy is used. However, management of seizures with antiepileptic medication polypharmacy may prove to be challenging to some primary care physicians. When the primary care physician feels that the needs of their patient exceed their own knowledge and experience, referral to a pediatric neurologist is warranted. In addition, referral to a comprehensive epilepsy center is warranted for the evaluation of treatment with vagal nerve stimulation or epilepsy surgery.

I. Management by Age

Ongoing

  • A comprehensive medical history of seizure type(s), frequency triggers and duration should be obtained
  • A complete physical and neurological exam, including evaluation if possible congenital anomalies and specific skin lesions, should be done
  • EEG A brain MRI is a standard part of the workup
  • Other studies, including genetic metabolic studies may be indicated
  • Carefully monitor levels, effectiveness and side effects of all medications
  • Watch for changes in intellectual skills and school progress
  • Ensure that all caregivers know what to do in the event of a seizure
  • Encourage as normal a lifestyle as possible
  • Advise to avoid life-endangering situations (scuba diving, parachuting, swimming or bathing in a tub alone, two-wheeled vehicles on busy roads, etc.)

Infancy (Birth to 1 year)

  • Refer to pediatric neurologist
  • Refer to early intervention programs and all cases to epilepsy/disability support groups
  • Discuss possibility of SSI enrollment

Adolescence and Adulthood (13 years and over)

Most people with epilepsy are cognitively normal and are able to self-support and thrive in the community without unusual support systems; the following should be considered only when appropriate, such as for persons with mental retardation.

  • Discuss operating a motor vehicle, a stove, dangerous machinery, and working at lights
  • Discuss long-term financial plans
  • Discuss alternative community living resources
  • Discuss community-supported employment opportunities
  • Monitor prevocational training and vocational activities

II. Standard Anticonvulsant Medications
Status Epilepticus

  • First choice: benzodiazepines: diazepam, Diastat, lorazepam, midazolam (may cause respiratory depression)
  • Second choice: fosphenytoin or phenytoin
  • Third choice: phenobarbital
  • Fourth choice: pentobarbital, versed and propofol

III. Management of Specific Influencing Factors

Food and Drink

  • Advise to limit caffeine intake
  • Advise to avoid alcohol consumption
  • Recommend adequate fluid intake
  • Recommend adequate protein and sugar

Environment

  • Limit abrupt, rhythmic stimuli
  • Avoid visual stimuli that flicker, flash or have strobing effect if patient has photoconvulsive response in EEG testing
  • Avoid extreme temperatures
  • Avoid rapid breathing
  • Ensure adequate rest and sleep
  • Avoid startling, tapping or sudden movements if startle seizures occur (rare)

IV. Psychosocial Considerations

Children with epilepsy and their parents face many psychosocial barriers because of the health implications of epilepsy, the stigma associated with epilepsy, and the unpredictability of the occurrence of seizures. They may experience:

Child

  • Feelings of guilt, shame, or anger
  • Feelings of helplessness and lack of control
  • Anxiety and depression
  • Behavioral problems, immaturity
  • Low academic achievement
  • Inappropriate levels of dependency
  • Deficiency in psychosocial skills
  • Isolation, few friends

Parents

  • Feelings of guilt, shame, or anger
  • Feelings of helplessness and lack of control
  • Anxiety and depression
  • Fear of intellectual deterioration, psychiatric disorders, drug side effects
  • Low expectations for the child
  • Overprotectiveness and strict limitations
  • Keeping the disorder a secret

Traditional counseling and psychiatric interventions have been of some help, yet there is a growing need for interventions that are child centered, skill based, and family focused. According to Dr. Mary Ann Lewis, et al., this program should:

  • Provide knowledge about epilepsy and seizures to both parents and child;
  • Teach skills related to decision making and communicating about seizures;
  • Involve children and parents as equal partners in gaining knowledge and skills;
  • Emphasize coping and parenting skill for a chronically ill child;
  • Enhance the chil, d, ‘s self-esteem and sense of responsibility; and,
  • Teach the child self-care skills and independence. (Lewis, et al. 1989)

The goal of therapy is optimal quality of life, and consideration should be given to the impact of the seizure disorder and of its treatment on peer relationships, schooling, employment, family, and other interrelated dynamics.

Several studies document that children and adolescents with epilepsy are at high risk for psychiatric problems which are often not identified. Educational/psychosocial programs should address mental health symptoms, as well as focusing upon quality of life. A description and critique of psychosocial interventions published in peer-reviewed journals (Wagner and Smith, 2005) is highly recommended for understanding the various methods and outcomes of all of the educational interventions published in the literature.

REFERENCES
back to top

Peer-reviewed Journal Articles/Academies

America’s Working Group on Status Epilepticus (1993). Treatment of Convulsive Status Epilepticus Journal of the American Medical Association, 270(7), 854-859.

(1998). Consensus Statements: Medical Management of Epilepsy. Wilder, B.J. (Section Ed.). Neurology, 51 (5 Suppl 4), S39-S43.

Lewis, M.A. et al. (1989). Randomized Trial of a Program to Enhance the Competencies of Children with Epilepsy. Epilepsia, 31(1), 100-109.

Mattson, R.H. (1998). Medical Management of Epilepsy in Adults. Neurology, 51(4), 15-20.

Nadkarni, S., LaJoie, J., Devinsky, O. (2005). Current Treatments of Epilepsy. Neurology, 64(3), 2-11.

Phillips, P. (1997). Current view of advances in epilepsy. Journal of the American Medical Association, 278(11), 883-886.

Scheuer, M.L., Pedley, T.A. (1990). The Evaluation and Treatment of Seizures New England Journal of Medicine, 323(21), 1468-1474.

Wagner, J.L. & Smith, G. (2005). Psychosocial Intervention in Pediatric Epilepsy: A Critique of the Literature. Epilepsy & Behavior, Article in Press, Corrected Proof, 1-11.

Special Interest Groups/Other Publications

American Epilepsy Society. et al. (2003). Living Well with Epilepsy. Report of the 2003 National Conference on Public Health and Epilepsy.

Baxter, P. (2005). Epilepsy and Sleep. Developmental Medicine and Child Neurology, 47(11), 723.

Berg, A.T. (2005). Special Education Needs of Children with Newly Diagnosed Epilepsy. Developmental Medicine and Child Neurology, 47(11), 749-753.

Besag, F.M.C., Nomayo, A., Pool, F. (2005). The Reactions of Parents who think that a Child is Dying in a Seizure –In Their Own Words. Epilepsy and Behavior, 7, 517-523.

Capute, A.J. & Accardo, P.J. (1996). Developmental Disabilities in Infancy and Childhood: Vol. 1. Neurodevelopmental Diagnosis and Treatment. Baltimore: Paul H. Brookes.

Capute, A.J. & Accardo, P.J. (1996). Developmental Disabilities in Infancy and Childhood: Vol 2. The Spectrum of Developmental Disabilities. Baltimore: Paul H. Brookes.

Datta, S.S., et al. (2005). Behaviour Problems in Children and Adolescents with Seizure Disorder: Associations and Risk Factors. Seizure, 14, 190-197.

Eastern Los Angeles Regional Center. (1998). Wellness Initiative<

Elliot, I.M., Lach, L., Smith, M.L. (2005). I Just Want to be Normal: A Qualitative Study Exploring how Children and Adolescents View the Impact of Intractable Epilepsy on their Quality of Life. Epilepsy & Behavior. (Article in Press), 1-15.

Gillian, F. (2002). Optimizing Health Outcomes in Active Epilepsy. Neurology, 58, 9-20.

Kelley, R.E. (2000). Status Epilepticus: Diagnosis and Management. Louisiana State University Medical Center. Accessed December 13, 2005.

Mitchell W.G. (1995). Long-Term Prognosis for Children with Epilepsy. Current Problems in Pediatrics, 25(3), 113-120.

National Information Center for Children and Youth with Disabilities. (1998). Epilepsy Fact Sheet. Accessed November 25, 1998.

Ogata, A., Amano, K. (2000). A Psychosocial Approach to Epileptic Patients. Epilepsia, 41(9), 36-38.

Piccioli, M. et al. (2005). Do Video Games Evoke Specific Types of Epileptic Seizures? Epilepsy and Behavior, 7, 524-530.

Reid. S. (2001). MOSES: An Educational Program for Patients with Epilepsy and Their Relatives. Epilepsia, 42(3), 76-80.

Sinclair, D. (1997). Status Epilepticus. University of Alberta Department of Pediatrics. Accessed February 5, 1999.

Smith Consultant Group & McGowan Consultants. (1998). Epilepsy. In M.L. Snyder(Ed.),Health and Wellness Reference Guide (pp. 197-214), TN: Neri Productions.

Strine, T.W. et al. (2005). Psychological Distress, Comorbidities, and Health Behaviors Among U.S. Adults with Seizures: Results from the 2002 National Health Interview Survey. Epilepsia, 47(7), 1133-1139.

Toffler, W.L., Fields, S.A. (1999). In M.R. Dambro (Ed.) Griffith’s 5 Minute Clinical Consult (pp. 978-979). Baltimore, MD: Lippincott Williams & Wilkins.

Tsang-Wai, R. et al. (2005). Outcomes in Pediatric Epilepsy: Seeing Through the Fog. Pediatric Neurology, 33, 244-250.

RESOURCES FOR FAMILIES
back to top

American Epilepsy Society

Wakeforest University Baptist Medical Center Epilepsy Information Service
800-642-0500

California Department of Developmental Services
916-654-1690

California Regional Centers
915-654-1958

Epilepsy Foundation of America
800-332-1000

Epilepsy Foundation of San Diego County
619-296-0161

Exceptional Parent Magazine
800-EPARENT

March of Dimes Birth Defects Foundation
916-922-1913

National Institute of Neurologic Disorders and Stroke

Cornell University Comprehensive Epilepsy Center

ADVISORY COMMITTEE
back to top

Theodore A. Kastner, M.D., M.S.
Felice Weber Parisi, M.D., M.P.H.
Mary Ann Lewis, Dr.P.H., R.N., F.A.A.N.
Jaime Mejlszenkier, M.D., F.A.A.N.
Terrance D. Wardinsky, M.D.
Larry Yin, M.D., M.S.P.H., F.A.A.P.

PUBLICATION INFORMATION
back to top

This document does not provide advice regarding medical diagnosis or treatment for any individual case, and any opinions or statements contained in this document are not intended to serve as a standard of medical care. Physicians are encouraged to view the considerations presented in this document in light of evolving scientific information. This document is not intended for use by the layperson. Reproduction of this document may be done with proper credit given to California Department of Developmental Services.