A Rare Childhood Seizure Disorder
Doose syndrome is a rare seizure disorder that begins during early childhood.
This condition is also called myoclonic astatic epilepsy and myoclonic atonic epilepsy.
Doose syndrome is considered a throw in of generalized epilepsy.
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The seizures of Doose syndrome may be difficult to manage with medication.
As children reach adolescence or adulthood, they may improve, and treatment might not be required anymore.
This article discusses Doose syndrome, its symptoms, and its causes.
It also goes over treatment and prognosis.
Recurrent seizures may begin weeks or months after the first seizure.
Associated Symptoms
Some children who have Doose syndrome may experience other effects besides seizures.
Complications
Doose syndrome can cause several health problems that have long-term effects.
Drop attacks are episodes of sudden falling that occur during or after a seizure.
This can result in major physical injuries.
Status epilepticusis a seizure episode that does not resolve on its own.
These seizures can interfere with breathing, and they require urgent medical intervention.
Status epilepticus is an uncommon complication of Doose syndrome.
What Causes Doose Syndrome?
Generalized seizures cause impairment of consciousness.
They can affect physical tone and movements, but they dont necessarily do so.
Genetics
Most children who are diagnosed with Doose syndrome have at least one family member with epilepsy.
Changes in one or more of these genes may cause or predispose children to Doose syndrome.
Electroencephalogram (EEG)
Most children who have recurrent seizures will have an EEG.
An EEG is a non-invasive brain wave test that measures the electrical activity of the brain in real-time.
During this test, your child would have small metal plates placed on their scalp.
The plates detect the electrical pattern of the brain.
Children with Doose syndrome have a certain pattern on their EEG.
These bursts of activity may occur frequently throughout the study.
The overall background brain activity may be normal or abnormal when the spikes are not present.
The brain structure as detected by imaging tests is typically completely normal in children who have Doose syndrome.
The results are expected to be normal in Doose syndrome.
How Doose Syndrome is Treated
This condition may be difficult to treat.
In fact, some AEDs used for treatment of focal seizures may actually worsen generalized seizures.
It is not completely clear why steroids may be beneficial.
This diet is extremely difficult to maintain, and it is not effective unless it is strictly followed.
That is why it is not usually considered a desirable approach unless medication is not effective for controlling seizures.
While it can help reduce seizure frequency, the ketogenic diet can cause elevated fat and cholesterol levels.
In some circumstances, epilepsy surgery may be considered for children who have Doose syndrome.
What Is the Prognosis for Doose Syndrome?
Doose syndrome is not known to impact life expectancy.
However, the frequency and duration of seizures can vary significantly from person to person.
Around 60% will have normal intellectual development, and 20% will have a mild developmental delay.
The remaining 20% will have moderate to severe developmental delay.
Children with this condition have a combination of different types of seizures that occur regularly.
The condition may be treated with anti-epilepsy drugs, steroids, or dietary management.
With early treatment, some children with Doose syndrome will outgrow the condition.
While some children may experience developmental delays, more than half will have normal intellectual development.
Results of the Pediatric Epilepsy Research Consortium survey.
2023;5(1).
