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Epilepsy Syndromes in Children

Epilepsy syndromes affect infants and children, and are characterized by a variety seizures and other symptoms such as developmental delays.

Epilepsy Syndromes in Children: What You Need to Know

  • Epilepsy syndromes are likely to be congenital (present at birth) or appear during childhood.

  • Epilepsy syndromes are defined either by a unique combination of symptoms or by the location (lobe) in the brain where the seizures originate.

  • Epilepsy may be treated with medication, and occasionally with diet therapy, nerve stimulation or surgery.

Infantile Spasms (West Syndrome)

Infantile spasms typically begin between 3 and 12 months of age and usually stop by the age of 2 to 4 years. The spasms appear as a sudden jerk or jolt followed by stiffening. Often the child’s arms fling outward and the knees pull up as the body bends forward.

Each seizure lasts only a second or two but recur close together in a series. Sometimes the spasms are mistaken for colic, but colic cramps do not typically occur in a series. The child is most likely to have the spasms just after waking up, but they can also occur — rarely — during sleep.

Infantile spasms are a particularly severe form of epilepsy that can lead to poor development, and children with these symptoms should be evaluated right away. Treatment for infantile spasms usually includes steroid therapy, certain anticonvulsant medications or a ketogenic diet.

Doose Syndrome (Myoclonic Astatic Epilepsy of Childhood)

Myoclonic astatic epilepsy (MAE), also known as Doose syndrome, is an epilepsy syndrome of early childhood, most commonly appearing between ages 1 and 5 and featuring generalized seizures. Children will experience drop attacks and staring seizures, sometimes associated with falls. MAE is idiopathic, meaning the cause is not yet known.

Although MAE seizures are often resistant to medication, anti-seizure drugs valproate and levetiracetam may be helpful. However, research has shown that the ketogenic diet and modified Atkins diet are often the most effective treatments.

Benign Rolandic Epilepsy (BRE)

Benign rolandic epilepsy, also known as BRE or benign epilepsy with centrotemporal spikes (BECTS), is an epilepsy syndrome affecting children. It accounts for about 15 percent of childhood epilepsy cases.

Seizures appear as twitching, numbness, or tingling of the child’s face or tongue, which may interfere with speech and cause drooling. Seizures spread and become generalized seizures. In many cases, BRE seizures are infrequent, and they usually occur only at night. These seizures typically last no more than two minutes, and the child remains fully conscious. BRE typically begins between ages 6 and 8, and is more likely to affect boys than girls.

Many children do not take any seizure medicines for BRE, and the seizures nearly always stop by early adolescence. If the child has seizures during the day, if seizures disrupt sleep at night, or if the child has a reading disability that may be associated with BRE, the doctor may prescribe levetiracetam or oxcarbazepine.

Rasmussen Syndrome

Rasmussen's syndrome (also known as Rasmussen’s encephalitis) appears to be an autoimmune process that causes one hemisphere of the brain to become inflamed and deteriorate.

Rasmussen's syndrome typically appears in children who are between 14 months and 14 years of age. Seizures are often the first symptoms to appear. Weakness and other neurological problems often begin 1 to 3 years after the seizures start.

Treatment can halt the inflammation, but cannot reverse the damage. Other disorders (including other forms of brain inflammation, also known as encephalitis) can resemble Rasmussen syndrome, so it is important for parents to consult a specialist.

The seizures associated with Rasmussen’s syndrome are extremely difficult or impossible to control with medication. In many children with the condition, the best treatment option is epilepsy surgery.

Lennox-Gastaut Syndrome

Typically beginning in children between the ages of 2 and 6, Lennox-Gastaut syndrome is idiopathic — it has no known cause — and is commonly found in children with brain development problems or acquired brain damage.

Children with Lennox-Gastaut syndrome have developmental delays and show a distinctive electroencephalogram (EEG) pattern with slow spike and waves.

The seizure types seen in Lennox-Gastaut syndrome include atonic, tonic, tonic-clonic and atypical absence seizures. Because atonic seizures cause children to fall to the ground, many children wear helmets to protect their teeth and face.

Management can be challenging and can include anticonvulsant medications, nerve stimulation and certain types of diet therapy.

Electrical Status Epilepticus of Sleep (ESES)

Electrical status epilepticus of sleep (ESES) describes seizure activity that becomes more frequent during sleep and is seen in more than half of a child’s sleep EEG.

ESES is found in school-age children, and it can affect different children in different ways and to varying degrees. Those who have ESES often, but not always, have seizures, and their types may vary. Children with ESES often have cognitive regression — a decline in their ability to learn and to do things they already know how to do.

This ongoing seizure activity can lead to difficulty speaking or understanding speech, behavioral concerns, attentional difficulties, or problems with movement of one or more parts of the body.

When the seizure activity is combined with another feature of ESES, it is often called continuous spike and wave during sleep (CSWS). There are other more specific syndromes such as Landau-Kleffner syndrome (LKS), in which children have ESES on their EEG and specifically have difficulty with language and understanding sounds.

Medications typically used to treat seizures can be used to treat ESES, but the doctor may need to add more therapies such as high dose benzodiazepines and steroids. Close monitoring of children’s cognitive function is essential.

Sturge-Weber Syndrome

Sturge-Weber syndrome (SWS) is a neurocutaneous disorder (one that affects the brain and skin) identifiable by the “port-wine stain” (known as an angioma) on the forehead area around the eye.

Children with SWS may experience seizures, weakness on one side of the body (hemiparesis), developmental delays and increased pressure in the eye (glaucoma).

Seizures occur in more than 80 percent of children diagnosed with SWS. Approximately 25 percent of those children achieve full seizure control with medication, 50 percent obtain partial seizure control, and 25 percent do not respond to medication. In severe cases, the doctor may recommend epilepsy surgery.

More Information About Sturge-Weber Syndrome from Johns Hopkins Medicine

Medical Breakthroughs, Transformative Care

The faculty and staff of the Hunter Nelson Sturge-Weber Center at Kennedy Krieger Institute are dedicated to providing the highest quality of comprehensive care for patients with Sturge-Weber syndrome and new research uncovering the cause of SWS.

Visit the Center.

Juvenile Myoclonic Epilepsy

People with juvenile myoclonic epilepsy (JME) have myoclonic seizures, characterized by small, rapid jerks of the arms, shoulders, or occasionally the legs. These usually occur soon after awakening. The myoclonic jerks sometimes are followed by a tonic-clonic seizure or tonic-clonic seizures can occur independently. Absence seizures also may occur, where the patient seems to “blank out” for a short period of time that can last from seconds to several minutes.

JME seizures may begin between late childhood and early adulthood, usually around the time of puberty. In most patients, seizures are well controlled with long-term medication.

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