Benign Rolandic Epilepsy
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Starts in children aged 3 - 13. Most common onset at age 9 - 10.
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Common, "mild" form of epilepsy, characterized by brief simple partial seizures involving the mouth and face ("twitching"), usually occurring at night or during the early morning hours. Is considered idiopathic.
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Child will be awake and aware that her face muscles are twitching, she'll gradually lose the ability to talk, and may drool. Or one side of the face may feel "paralysed". Occasionally, it will generalize to cause a tonic seizure on that side of the body, or a full tonic-clonic seizure.
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Seizures may occur infrequently or many times a day.
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Is considered "benign" because it is usually outgrown by adolescence, whether treated or not. Also, seizures usually occur when child is still in bed, or at least within the safety of her home. Finally, child is generally not negatively affected - no developmental or learning challenges are associated with this syndrome.
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"Rolandic" refers to the Rolandic or motor strip, where the seizure focus is located. Medications may or may not be prescribed. Tegretol is first line drug, followed by valproic acid. Usually medications are considered when the seizures are very frequent, bothersome to the child, or the parent worries about nocturnal seizures.
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Medications are generally tapered off once the child reaches puberty or after a seizure-free period of about three years.
Juvenile Myoclonic Epilepsy
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Starts in late childhood or early adolescence, often about the onset of puberty. Characteristic feature is mild myoclonic jerks, most commonly as person is going to sleep or awakening in the morning.
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Adolescent will describe jerking of the arms or legs, or a feeling of being very "jumpy". Often patients will set their alarm clocks to go off early, and then stay in bed for half-hour to an hour until the jumpiness wears off.
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Occasionally, person experiences a clonic or tonic-clonic seizure. Absence seizures may also occur.
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The EEG between seizures shows a fast, multiple- or double-spike pattern followed by slow waves, with fast rapid spiking occurring during the jerks.
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Treatment includes: ACTH (a form of steroid that is given twice a day by intramuscular injection; initially in hospital, then nurse comes to home; many risky side effects); vigabatrin; "benzos" such as Valium or clonazepam; or valproic acid.
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Epilepsy, in the form of other seizure types following the disappearance of infantile spasms, occurs in about 55 - 60 % of children. Lennox-Gastaut syndrome and epilepsy with complex partial seizures are the most common forms.
Lennox-Gastaut Syndrome
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One of the severest forms of childhood epilepsy.
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Characterized by two or more seizure types, one of which is atonic (drop seizures), by a particular EEG pattern of diffuse spike or poly-spike and slow waves, and by "mental retardation" (occurring prior to seizure onset in 20-60 % of cases). Other seizure types include: myoclonic, tonic, tonic-clonic, atypical absence. Child will usually wear a helmet with face guard.
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Nonconvulsive and tonic status may occur.
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Along with "mental retardation", may be disturbances of behaviour and personality.
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Usually begins between the ages of 2 and 6; in more than 1/3 of cases, the child previously experienced infantile spasms.
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No single cause, but commonly arises in children with developmental problems of the brain or acquired brain damage
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Small percentage of these children appears "normal" before seizures begin and have a later age of onset. Important to look for a degenerative process that may be causing the seizures. Cryptogenic cases may have a somewhat better prognosis than symptomatic cases.
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Often multiple medications (leading to high incidence of side effects). These include: valproic acid; lamotrigine; vigabatrin. Ketogenic diet has been shown to be quite effective. Possibly vagus nerve stimulation. A particular form of surgery, corpus callosum sectioning, is under investigation with these children.
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These children make up large proportion of the intractable seizure group.
Neonatal Seizures
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Typical absence and generalized tonic-clonic are not seen in newborns.
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Any type of "bizarre" or unusual transient event in the neonatal period may be a seizure, especially if "stereotyped" and periodically recurring. .
- Differences between epileptic seizures in newborn babies vs. older children probably due to incomplete development of the baby's brain.
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Child with a first seizure related to fever does not necessarily require brain scans or EEG. A lumbar puncture will be ordered if meningitis or encephalitis is suspected.
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Febrile seizures are thought to have a genetic basis.
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25 - 30 % of children who have had one febrile seizure will have another. If seizure occurred in first 18 months of life, within first hour of illness with only a moderate temperature, of if there is a family history, chances of having another febrile seizure may be greater (over 80 %).
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No evidence that recurrent febrile seizures damage the brain or lead to "mental retardation" or learning disabilities.
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Phenobarbital is the only drug that can safely and effectively treat febrile convulsions. Tegretol and Dilantin won't work. Valproic acid is unsafe in very young children due to risk of fatal liver disease. Or diazepam (Valium) can be given by mouth or rectally when the child has a fever to decrease chance of another febrile seizure occurring (but causes sleepiness and irritability).
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Often AED's aren't used. Parents are told to treat fevers or conditions that can cause a fever (e.g. ear infections) very aggressively (e.g. using Tempra or Tylenol).
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Of children who have had a febrile seizure, 98 % will not develop epilepsy. Risk factors for developing epilepsy following one febrile seizure include:
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if the first febrile seizure was prolonged (more than 15 minutes); if the seizure was one-sided or focal; of if there were two or more seizures during the initial episode;
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if there is a family history of epilepsy;
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if child has a neurological disorder (e.g. cerebral palsy or developmental delay)
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If there are none of these risk factors, the child has a 1 % chance of developing epilepsy; with all three risk factors, the child has a 10 % chance. No evidence that the risk of epilepsy increases even with more febrile seizures; nor does taking medication after a febrile seizure reduce the risk of developing epilepsy later on.
Special Conditions that cause Epilepsy
Landau Kleffner Syndrome (also called Acquired Epileptic Aphasia)
Rasmussen's Syndrome (also called Rasmussen's Encephalitis)
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Rare form of progressive unilateral seizures and associated weakness on one side of the body. Mental functioning will decline.
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May be a viral cause, although currently unknown. Believed to be due to an autoimmune process in which the body destroys its own brain cells.
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Often sudden onset of intense partial seizures that do not spread. Or it may begin with generalized or tonic or unilateral tonic-clonic seizures, frequently evolving into "epilepsia partialis continua" (continuous focal epilepsy).
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No effective medication to stop the condition's progression. AED's are rarely effective. Only effective treatment is hemispherectomy (removal of one side of the brain). If done early, it usually stops seizures and permits normal intellectual development. After surgery, there is paralysis, but without surgery the consequences are worse (progressive intellectual deterioration, increased seizures, continued weakness or loss of movement).
Degenerative Diseases (e.g. Tay Sachs, Batten's Disease, Lafora Disease)
References:
Aicardi, Jean Epilepsy In Children (2"¡ ed.) Lippincott-Raven: New York, 1996. Appleton, R.E. "The Landau Kleffner Syndrome" unknown source.
Freeman, John, Eileen Vining and Diana Pillas Seizures and Epilepsy in Childhood: A Guide for Parents Johns Hopkins Press: Baltimore, 1997.
Snead, O. Carter, "Pediatric Aspects of Epilepsy" unknown source.
Benign Rolandic Epilepsy Fact Sheet, Epilepsy Toronto, 1999.
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