Doose Syndrome Treatment and Prognosis

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Table of Contents

Symptoms

Causes

Diagnosis

Treatment

Prognosis

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 type of generalized epilepsy. 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.

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Doose Syndrome Symptoms

The first seizure of Doose syndrome generally begins between 7 months and 6 years old. The condition affects children who were previously healthy and who attained developmental milestones on time (such as walking, talking, and socializing). Recurrent seizures may begin weeks or months after the first seizure.

Seizure types that occur in Doose syndrome include:

Children who have Doose syndrome generally experience several types of seizures on a regular basis. This condition ranges in severity, with some children having many seizures every day, and some having a few seizures per week.

Associated Symptoms

Some children who have Doose syndrome may experience other effects besides seizures.

Some children with Doose syndrome haveataxia(trouble with coordination),dysarthria(unclear speech), or features ofautism(difficulty expressing themselves and interacting with others).

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.

Developmental regression, which is loss of physical or cognitive abilities that have already developed, can occur as well.

What Causes Doose Syndrome?

There are not known risk factors that are definitively linked with Doose syndrome and there is not usually a specific cause or trigger for the individual seizures. However, common seizure triggers, including fevers and fatigue, can trigger episodes.Photosensitive seizures, which occur in response to flashing lights, can occur as well.

The seizures that occur in Doose syndrome are generalized seizures, which means that they begin with abnormal nerve activity throughout the whole brain. This is in contrast to focal seizures, which begin with abnormal nerve activity in a small region of the brain, and may spread throughout the whole brain.

Generalized seizures cause impairment of consciousness. They can affect physical tone and movements, but they don’t necessarily do so. For example, absence seizures don’t affect muscle movement, but myoclonic seizures and atonic seizures do—and they all cause impairment of consciousness and a lack of awareness.

Genetics

Most children who are diagnosed with Doose syndrome have at least one family member with epilepsy. The condition has been thought to possibly have a genetic cause, but there is not a clear-cut hereditary pattern of inheritance.

Several genes have been linked to the condition, including SCN1A, SCN1B, GABRG2, CHD2, and SLC6A1. Changes in one or more of these genes may cause or predispose children to Doose syndrome.

Certain features, including the seizure pattern, normal childhood development, a family history of epilepsy, and diagnostic test results, can identify Doose syndrome.

With Doose syndrome, children usually have a normal physical examination, which is not expected to show signs of physical deficits or neurophysiological problems.

Electroencephalogram (EEG)

Most children who have recurrent seizures will have an EEG. This test generally lasts for about half an hour, although an extended EEG or an overnight EEG can be done as well.

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.

Each plate is connected to a wire that sends a signal to a computer so the computer can read the electrical brain wave pattern.

What to Expect From an EEG

Children with Doose syndrome have a certain pattern on their EEG. The test would show generalized (throughout the brain) spike wave activity at a frequency of 2 to 5 Hertz (Hz).

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.

Diagnostic Studies

In some instances, other tests such as blood tests, urine tests, and a lumbar puncture may be needed to rule out other causes of epilepsy. The results are expected to be normal in Doose syndrome.

How Doose Syndrome is Treated

This condition may be difficult to treat. The anti-epilepsy drugs (AEDs) used for generalized epilepsy are not the same as those used for focal epilepsy. In fact, some AEDs used for treatment of focal seizures may actually worsen generalized seizures.

AEDs commonly used in treatment of Doose syndrome include:

Some children may experience improvement of seizures with one AED (monotherapy), and sometimes a combination of these medications may be necessary.

Steroid Treatments

In addition to AEDs, other treatments may also be used for the management of seizures in Doose syndrome. Steroid medications, including adrenocorticotrophic hormone (ACTH), methylprednisolone, prednisone, or dexamethasone have been beneficial for some children who have this condition.

It is not completely clear why steroids may be beneficial. Steroids decrease inflammation and may alter hormone levels, and it has been suggested that the improvement could be related to one of these two actions.

While neither hormones nor inflammation has been directly linked to Doose syndrome, some children nevertheless experience improvement after using these treatments.

Dietary Management

The ketogenic diet is another approach that has been considered one of the strategies for managing seizures in refractory epilepsy, which is epilepsy that is not well controlled with AED medications.

2:13The Ketogenic Diet and Epilepsy

2:13

The Ketogenic Diet and Epilepsy

The ketogenic diet is a high fat, adequate protein, and extremely low carbohydrate diet. It is believed to control seizures through a physiological process called ketosis, in which the body forms ketones due to a type of metabolic breakdown that occurs in the absence of carbohydrate intake.

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.

Children who are on this diet may crave sugar, bread, or pasta—and if they can physically reach for food, young children often are not able to follow the diet as strictly as needed.

While it can help reduce seizure frequency, the ketogenic diet can cause elevated fat and cholesterol levels.

If your child is prescribed the ketogenic diet, it is a good idea to find a support group of other parents whose children are following the ketogenic diet so you can share recipes and strategies.

How to Use the Ketogenic Diet

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.

With early diagnosis and good treatment response, around 68% of children with Doose syndrome will achieve remission and stop having seizures altogether. 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.

Factors associated with a poorer prognosis include:

Summary

Doose syndrome is a type of epilepsy that begins in early childhood. 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.

4 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Nickels K, Thibert R, Rau S, Demarest S, Wirrell E, Kossoff EH, et al.How do we diagnose and treat epilepsy with myoclonic-atonic seizures (Doose syndrome)? Results of the Pediatric Epilepsy Research Consortium survey. Epilepsy Res. 2018 Aug;144:14-19. doi:10.1016/j.eplepsyres.2018.04.010Kanai S, Okanishi T, Nishimura M, Iijima K, Yokota T, Yamazoe T et al.Successful corpus callosotomy for Doose syndrome. Brain Dev. 2017 Nov;39(10):882-885. doi: 10.1016/j.braindev.2017.06.001Doose Syndrome Epilepsy Alliance.What is the long term prognosis?Ahakoud M, El Mouhi H, Belghiti HD, Bouguenouch L, Chaouki S.A mini review of Doose syndrome: Clinical manifestations, diagnosis, and treatment.Mor J Public Health. 2023;5(1).

4 Sources

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Nickels K, Thibert R, Rau S, Demarest S, Wirrell E, Kossoff EH, et al.How do we diagnose and treat epilepsy with myoclonic-atonic seizures (Doose syndrome)? Results of the Pediatric Epilepsy Research Consortium survey. Epilepsy Res. 2018 Aug;144:14-19. doi:10.1016/j.eplepsyres.2018.04.010Kanai S, Okanishi T, Nishimura M, Iijima K, Yokota T, Yamazoe T et al.Successful corpus callosotomy for Doose syndrome. Brain Dev. 2017 Nov;39(10):882-885. doi: 10.1016/j.braindev.2017.06.001Doose Syndrome Epilepsy Alliance.What is the long term prognosis?Ahakoud M, El Mouhi H, Belghiti HD, Bouguenouch L, Chaouki S.A mini review of Doose syndrome: Clinical manifestations, diagnosis, and treatment.Mor J Public Health. 2023;5(1).

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.

Nickels K, Thibert R, Rau S, Demarest S, Wirrell E, Kossoff EH, et al.How do we diagnose and treat epilepsy with myoclonic-atonic seizures (Doose syndrome)? Results of the Pediatric Epilepsy Research Consortium survey. Epilepsy Res. 2018 Aug;144:14-19. doi:10.1016/j.eplepsyres.2018.04.010Kanai S, Okanishi T, Nishimura M, Iijima K, Yokota T, Yamazoe T et al.Successful corpus callosotomy for Doose syndrome. Brain Dev. 2017 Nov;39(10):882-885. doi: 10.1016/j.braindev.2017.06.001Doose Syndrome Epilepsy Alliance.What is the long term prognosis?Ahakoud M, El Mouhi H, Belghiti HD, Bouguenouch L, Chaouki S.A mini review of Doose syndrome: Clinical manifestations, diagnosis, and treatment.Mor J Public Health. 2023;5(1).

Nickels K, Thibert R, Rau S, Demarest S, Wirrell E, Kossoff EH, et al.How do we diagnose and treat epilepsy with myoclonic-atonic seizures (Doose syndrome)? Results of the Pediatric Epilepsy Research Consortium survey. Epilepsy Res. 2018 Aug;144:14-19. doi:10.1016/j.eplepsyres.2018.04.010

Kanai S, Okanishi T, Nishimura M, Iijima K, Yokota T, Yamazoe T et al.Successful corpus callosotomy for Doose syndrome. Brain Dev. 2017 Nov;39(10):882-885. doi: 10.1016/j.braindev.2017.06.001

Doose Syndrome Epilepsy Alliance.What is the long term prognosis?

Ahakoud M, El Mouhi H, Belghiti HD, Bouguenouch L, Chaouki S.A mini review of Doose syndrome: Clinical manifestations, diagnosis, and treatment.Mor J Public Health. 2023;5(1).

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