By: Dr. Sharon Elza Raju
What is Doose Syndrome?
Doose Syndrome is a very rare type of seizure that affects children during early childhood. It was initially known as Myoclonic Astatic Epilepsy and Myoclonic Atonic Epilepsy.
Back in 1989, the International League Against Epilepsy (ILAE) initially classified it as symptomatic generalized epilepsy. Considering it to be a much-generalized form of epilepsy, managing it with medication seems fairly difficult.
When a child experiences myoclonic-atonic seizures, the child could go limp. Children with Doose’s Syndrome could have other types of seizures as well, namely:
- Tonic-clonic seizures
- Absence seizures
- Atonic seizures
- Non-convulsive status epilepticus
Prevalence:
As mentioned, it is a very rare kind of epilepsy, it usually occurs in 1-2 children out of 100 children who are diagnosed with epilepsy.
This could occur in children who have a family history of epilepsy or early onset, usually between the ages of one and five.
Compared to girls, boys are more likely to have Doose Syndrome.
Symptoms:
This type of seizure could happen either once or hundreds of times a day. More often it happens during the day time and children who experience this would need constant supervision.
These could cause other health issues which involve difficulty in learning and development delays.
The condition affects children who have had a healthy lifestyle and have attained a developmental milestone.
It can, in fact, cause a number of other associated symptoms, for example having trouble with coordination, experiencing difficulty expressing themselves, and unclear speech.
Complications:
A child suffering from Doose syndrome would have long-term effects on his or her health. It could cause physical injuries, could cause breathing issues, and could also require immediate medical intervention.
Diagnosis:
Usually, a child exhibiting these symptoms would have a family history associated with epilepsy.
Doose syndrome can be diagnosed using diagnostic test results as the children would usually have a normal physical examination.
- Electroencephalogram (EEG) :
An EEG test usually lasts for an hour and is a noninvasive brain wave test used to measure the electrical activity of the child’s brain in real time. This test usually requires small metal plates to be placed on the child’s scalp and this would help in detecting the electrical pattern of the child’s brain. A spike would be observed in children with Doose syndrome (Frequency of 2 to 5 Hertz).
- Diagnostic Studies:
Other types of Brain Imaging could be used as a diagnostic tool, such as a CT scan or an MRI of the brain. Other medical tests would include blood work and urine analysis and if advised by the doctor, a lumbar puncture would be required to rule out any other causes of epilepsy.
Treatment:
- Due to the severity of the syndrome, it is fairly difficult to treat it. The anti-epilepsy drugs which are used to treat focal epilepsy are not used in this case.
- Some of the commonly used Anti-epileptic drugs include Depakote, Lamictal, and Keppra.
- In addition to this, there are a few other treatment options including steroidal medications like ACTH (Adrenocorticotrophic hormone), Prednisone, or dexamethasone.
- Steroids are used to decrease inflammation and could affect hormonal levels and it is suggested that improvements are related to these actions.
- Maintaining a ketogenic diet is considered one of the strategies that could help.
Conclusion:
Doose Syndrome is a very rare type of seizure that affects children during early childhood. It was initially known as Myoclonic Astatic Epilepsy and Myoclonic Atonic Epilepsy. Children with Doose syndrome can have multiple seizures a day, affecting learning and developmental milestones. Doose syndrome is difficult to treat but options such as anticonvulsant medication, steroidal medications, and the ketogenic diet can help.
Resources:
Cincinnati Children’s Hospital (2022). Doose Syndrome. Cincinnati Children’s Hospital. Retrieved from: https://www.cincinnatichildrens.org/health/d/doose-syndrome
Children’s Health (2022). Pediatric Doose Syndrome. Children’s Health. Retrieved from: https://www.childrens.com/specialties-services/conditions/doose-syndrome
Doose Syndrome Epilepsy Alliance (n.d.) What is Doose syndrome? Doose Syndrome Epilepsy Alliance. Retrieved from: https://doosesyndrome.org/parents-and-caregivers/what-is-doose-syndrome/
Epilepsy Queensland (2021). Doose Syndrome. Epilepsy Queensland. Retrieved from: https://epilepsyqueensland.com.au/about-epilepsy-epilepsy-queensland/epilepsy-syndromes/doose-syndrome/
Estiphan, T., Joshi, C. (2022). Epilepsy with Myoclonic-Atonic Seizures (Doose Syndrome). Child Neurology Foundation. Retrieved from: https://www.childneurologyfoundation.org/disorder/epilepsy-with-myoclonic-atonic-seizures-doose-syndrome/
Kelley, S. A., & Kossoff, E. H. (2010). Doose syndrome (myoclonic-astatic epilepsy): 40 years of progress. Developmental medicine and child neurology, 52(11), 988–993. https://doi.org/10.1111/j.1469-8749.2010.03744.x
Moawad, H. and Metrus, N. (2022). An Overview of Doose syndrome: A Rare Childhood Seizure Disorder. VeryWell Health. Retrieved from: https://www.verywellhealth.com/doose-syndrome-4772143
Nickels, K., Kossoff, E. H., Eschbach, K., & Joshi, C. (2021). Epilepsy with myoclonic-atonic seizures (Doose syndrome): Clarification of diagnosis and treatment options through a large retrospective multicenter cohort. Epilepsia, 62(1), 120–127. https://doi.org/10.1111/epi.16752
