Causes and Types
CAE is caused by abnormalities in the thalamus region of the brain which controls awareness and alertness. There are two main types – common childhood absence epilepsy and uncommon childhood absence epilepsy. Common CAE involves typical staring spells several times a day and is responsive to medications. Uncommon CAE has additional seizure types or is not well controlled with medications.
Medical Treatment
The first line treatment for CAE involves use of anti-seizure medications from a class called ethosuximide. Some commonly used brand names include Zarontin and Emeside. These medications work by calming down the abnormal electrical signals in the thalamus region of the brain. In majority of children, ethosuximide is very effective in reducing or eliminating seizure episodes. The medication needs to be taken everyday as prescribed by the doctor to maintain effective levels in the blood.
The starting dose is low and slowly increased based on the child’s response and any side effects. Common side effects seen are nausea, vomiting, loss of appetite and abdominal pain which are usually mild. Blood tests need to be done periodically to monitor medication levels and check for any side effects on the blood or liver. In about 10-20% of children who do not respond well to ethosuximide, other antiepileptic drugs like valproate or lamotrigine may be added or substituted.
Ketogenic Diet
For some children who fail two or more anti-seizure medication trials, an alternative treatment approach is the ketogenic diet. This special high fat, low carbohydrate and protein diet shifts the body’s metabolism to produce ketone bodies which have anticonvulsant effects. Studies have shown the ketogenic diet to be effective in reducing seizures in about 50% of children with CAE.
The Childhood Absence Epilepsy Treatment diet needs to be strictly followed under dietary supervision in the hospital initially. Potential side effects can include constipation, fatigue, dehydration, kidney stones which are manageable. Long term adherence to the strict diet can be challenging for some families. It is generally continued for 2-3 years after which medications are slowly reintroduced to see if seizures remain controlled.
Surgery
In very rare and refractory cases where seizures are not controlled despite trials of multiple antiepileptic drugs and the ketogenic diet, surgery may be considered. The surgery aims to remove or disconnect the specific areas of the brain that are causing the seizure disorder. Various techniques used include focal cortical resection, multiple subpial transections or corpus callosotomy. Successful seizure control is achieved in 50-60% of children post surgery but it also carries risks of neurological deficits. Hence, surgery is only pursued in carefully selected cases under an epilepsy surgery team.
Prognosis
With proper diagnosis and standardized treatment approach, the long term prognosis for childhood absence epilepsy is excellent. Approximately 60-70% of children outgrow the condition by late adolescence as the brain continues to mature. Anti-seizure medications can usually be gradually discontinued 2-4 years after the last seizure episode. Close monitoring is required as there is small risk (5-10%) of recurrence in later life. Overall, majority of children with CAE go on to live normal, active and seizure-free lives into adulthood.
*Note:
1. Source: Coherent Market Insights, Public sources, Desk research
2. We have leveraged AI tools to mine information and compile it
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