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Developmental and epileptic encephalopathy (DEE) refers to a group of severe types of epilepsy that begin in infancy or childhood. This page discusses the causes of developmental and epileptic encephalopathies and how they are diagnosed and treated.
What is developmental and epileptic encephalopathy (DEE)?
Developmental and epileptic encephalopathy (DEE) is an umbrella term for a group of severe types of epilepsy. Seizures caused by a DEE usually begin in infancy or childhood. These seizures often do not respond to anti-seizure medications.
Developmental and epileptic encephalopathies affect the way the brain functions. Children who have DEE often experience intellectual and developmental disabilities as well.
Examples of common developmental and epileptic encephalopathies include:
- West syndrome (infantile seizures)
- Dravet syndrome
- Lennox-Gastaut syndrome
- Developmental and Epileptic Encephalopathy with Spike-Wave Activation in Sleep (DEE-SWAS, previously known as Landau-Kleffner syndrome)
- Ohtahara syndrome
- Rasmussen syndrome
- Epilepsy with myoclonic-atonic seizures
What causes DEE?
Developmental and epileptic encephalopathies are most often caused by changes (variants) in genes. They may also be caused by structural, metabolic, or immune disorders. In some cases, the cause of a DEE is unknown.
How are developmental and epileptic encephalopathies diagnosed?
DEEs are often diagnosed through more than one method, which can include:
- Genetic testing
- Imaging such as MRI or PET scan
- EEG and VEEG
- Discussion with a clinician about seizure frequency and types
How is DEE treated?
The treatment for developmental and epileptic encephalopathies may be a combination of options. While anti-seizure medications are usually the first choice to treat a seizure disorder, most types of DEE are resistant to these medications and therefore need different treatment. Some of these treatment options may include:
A vagus nerve stimulator, or VNS, is a device that is implanted in the chest wall. A VNS can help prevent seizures and reduce their length by sending regular, mild pulses of electrical energy to the brain along the cranial nerve in the neck.
A VNS is implanted during a 50- to 90-minute surgical procedure under general anesthesia. The stimulator is then programmed to deliver electrical impulses 24 hours a day. Side effects are usually mild, and may include tingling or mild discomfort in the neck, hoarseness, increased coughing, and difficulty swallowing.
Deep brain stimulation, or DBS, involves implanting a device known as a neurostimulator in the abdomen or chest. A wire, called a lead, is also implanted in the brain. The neurostimulator delivers constant, low-voltage electrical impulses to targeted areas of the brain. DBS can help reduce the frequency and severity of seizures that are drug-resistant. It is also used to treat complex movement disorders like dystonia.
There are a variety of neurosurgeries that can help treat or manage seizures caused by developmental and epileptic encephalopathies. These include:
- Resective epilepsy surgery, where the portion of the brain that causes the seizures is removed
- Hemispherectomy, performed on people with abnormalities in one hemisphere of the brain
- Corpus callosotomy, in which the bundle of nerves that joins the two hemispheres of the brain is cut
- Subdural electrode insertion, where electrodes are placed directly in contact with the brain in order to pinpoint the exact region of the brain that is causing seizures
Distinct from the fad “keto diet,” a medical ketogenic diet emphasizes low carbohydrates, high fat, and adequate protein to put the body into a state called ketosis. Ketosis forces the body to use fat, rather than glucose, as fuel. Being in a state of ketosis reduces seizure activity in some children.
Why Choose Gillette Children's for DEE Care
When you come to Gillette Children’s for epilepsy care, you benefit from the foremost experts in pediatric neurology, neurosurgery, complex care, and numerous other specialties.
Our designation as a Level 3 Epilepsy Center by the National Association of Epilepsy Centers (NAEC) means that Gillette Children’s has demonstrated excellence in epilepsy care and management. From initial diagnosis of seizure disorders in our Seizure Evaluation clinic to long-term management of epilepsy and its associated effects, the pediatric neurology experts at Gillette Children’s are here for you and your family.
Gillette's EEG lab is accredited by the American Board of Registration of Electroencephalographic Technologists (ABRET). This accreditation highlights our neurodiagnostic lab's independent review against national standards for equipment, staffing qualifications, safety protocols, and quality of recordings/interpretations, and has been verified to meet those standards.
Additionally, the advanced capabilities of our imaging services include 3 Tesla MRI, a scanner that is twice as powerful as standard MRI scanners (which operate at 1.5 Teslas).
3T MRI offers much greater detail in imaging results, which allows providers to get a more thorough look at the brain, blood vessels, and tissue of children who have complex epilepsy.
Our Keto Diet clinic—staffed by our experts in neurology, complex care, nursing, and pharmacy services—assists with the evaluation, implementation, and ongoing monitoring support for pediatric patients recommended for the ketogenic diet to manage and reduce their seizures.
From initial hospitalization to begin the diet, to family education about keto-compliant nutrition and medicine, to continuous medical monitoring and adjustment, the Keto Diet clinic at Gillette is here to support patients and their families as they adapt to a new way of eating for epilepsy management.
Gillette has a team of psychologists and neuropsychologists who have extensive experience with medically complex children. This includes evaluation and treatment for mental health concerns (anxiety, depression, etc.) and clarification of specific intellectual disabilities that can guide school and therapy resources in the future.
Many children with DEEs also have medical conditions beyond epilepsy. This may include cerebral palsy, genetic or metabolic disorders, and other conditions that require involvement of different medical or surgical conditions. Gillette Children's has teams of providers trained in complex care, physical medicine & rehabilitation, orthopedic surgery, neurosurgery, and other subspecialties that can evaluate and treat these conditions alongside the treatment your child receives for epilepsy.
