Most people with absence seizures are told their seizures will have a good response to medications and may even go away in adolescence or adulthood. However, this is not the case for all. For some, the seizures respond to medications, but return with each attempt to wean medications. For others, the absence seizures continue, despite trials of numerous anti-seizure medications. This often leads to questions, such as “Have I been treated with the best medications,” “Have my doctors made the wrong diagnosis” and, most importantly, “Why am I still having absence seizures?”
What are absence seizures?
Absence seizures are typically very brief seizures, lasting less than 20 seconds. The seizures happen without warning. During a simple absence seizure, the person stares and has decreased responsiveness. During a complex absence seizure, abnormal movements, most commonly blinking repetitively, are also seen. Other movements may include brief jerking of the arm and shoulder muscles or mouth movements. Afterwards, the person is completely alert and resumes the previous activity. These seizures often occur many times throughout the day.
Who has absence seizures?
Specific seizure type or types, age of onset, severity and frequency of seizures, cause of seizures, and certain patterns on the EEG may suggest certain type of epilepsy, or epilepsy syndrome. Absence seizures commonly occur in those with normal development and cognition, although they may have some learning and behavioral difficulties and are seen in several epilepsy syndromes. Determining the epilepsy syndrome, when possible, is helpful because it provides information on the most helpful treatments and prognosis for remission.
Childhood onset absence seizures are most commonly seen in childhood absence epilepsy (CAE). CAE occurs in children with normal development and often runs in families. The prognosis is generally favorable, with excellent response to anti-seizure medications and resolution of seizures in mid-adolescence. However, this is not the outcome for all those with absence seizures. There are additional epilepsy syndromes in which absence seizures occur, but the seizures are less likely to respond to medications and/or spontaneously remit.
Who else gets absence seizures?
Absence seizures that begin age 10-16 years are likely related to juvenile absence epilepsy (JAE). The appearance of seizures in JAE is very similar to those in CAE, but may occur less often and last longer. Generalized tonic clonic seizures are also more likely to occur. The response to medications in JAE is usually very good, but the likelihood of remission from epilepsy is low and the seizures are often life long.
In some children previously diagnosed with CAE, new seizure types occur as adolescence approaches. This is commonly seen in juvenile myoclonic epilepsy (JME). The absence seizures may improve with time, but myoclonic seizures and generalized tonic clonic seizures are frequent. The majority of people with JME respond well to anti-seizure medications, but, unlike CAE, spontaneous remission in JME is rare and only 10% are eventually able to discontinue medications.
In childhood absence epilepsy, juvenile absence epilepsy, and juvenile myoclonic epilepsy, the response to medications is usually very good. However, it is important to understand that this is not the case for all. Unfortunately, a minority of children and adolescents have absence seizures in spite of multiple trials of different medications. This may warrant further evaluation to ensure the correct epilepsy syndrome has been diagnosed. Furthermore, just as later onset absence seizures are suggestive of a poorer prognosis, early onset absence epilepsy is also more difficult to treat. Absence seizures that begin prior to age 3 years may be due to an underlying genetic or metabolic cause and should be further evaluated.[1, 2]
In addition, there are some absence epilepsy syndromes that present during childhood, but there are special features associated with the absence seizures that suggest a poor prognosis. For example, some children have brief jerks of their shoulders and arms at the same time as the absence seizure, called myoclonic absence seizures. Some may have twitching type movements around the mouth, called perioral myoclonia. Uncommonly, some people with absence seizures may also have very rapid blinking and twitching of the eyelid whenever they close their eyes, called eyelid myoclonia. When these special features are present, the epilepsy is typically poorly responsive to medications and remission from epilepsy in adulthood is rare.
What can I do to improve my seizure control?
For those experiencing difficult to treat absence seizures, additional treatment options exist. The mainstay of treatment continues to be combinations of anti-seizure medications, including zonisamide, valproic acid, topiramate, zonisamide, lamotrigine, levetiracetam, and benzodiazepines. As with all anti-seizure medications, it is important to take medications regularly and not miss doses. It is also important to avoid seizure triggers, such as sleep deprivation.
In addition, dietary therapy with the ketogenic diet, modified Atkins diet, or low glycemic index diet can be helpful in significantly reducing the frequency of seizures. These are prescription diets are very high in fat and low in carbohydrate to mimic the fasting state, causing the body to produce ketones. Although strict adherence to the diet is necessary and patients must be carefully monitored by the epilepsy physician and dietitian, greater than 50% reduction in absence seizures can be seen in over half of those treated.
Therefore, although absence seizures are typically thought to be easily treated, some with absence seizures may continue to experience symptoms even with appropriate medication therapies. However, with avoidance of seizure triggers, excellent compliance to medications, and dietary therapy, patients with difficult to treat absence seizures may see significant improvement in seizure control. They may even be able to say, “I am no longer having absence seizures.”
1. Kaneko, S., H. Iwasa, and M. Okada, Genetic identifiers of epilepsy. Epilepsia, 2002. 43(Suppl 9): p. 16-20.
2. Suls, A., S. Mullen, Y. Weber, K. Verhaert, B. Ceulemans, R. Guerrini, T. Wuttke, A. Salvo-Vargas, L. Deprez, L. Claes, A. Jordanova, S. Berkovic, H. Lerche, P. De Jonghe, and I. Scheffer, Early-onset absence epilepsy caused by mutations in the glucose transporter GLUT1. Ann Neurol., 2009. 66(3): p. 415-9.
3. Groomes, L., P. Pyzik, Z. Turner, J. Dorward, V. Goode, and E. Kossoff, Do patients with absence epilepsy respond to ketogenic diets? J. 26(2): p. 160-5. Epub 2010 Jul 20.
To learn more about the different Types of Seizures, please visit: http://my.epilepsy.com/epilepsy/types_seizures
Katherine Nickels, MD
Last Reviewed: 9/15/11
Article from the September 2011 Epilepsy.com Spotlight Newsletter. Other articles in this issue inclue:
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