Epilepsy Syndromes

There are many different types of epilepsy syndromes, due to abnormal brain activity resulting in wide variety of seizure types and syndromes.

Epilepsy syndromes are often classified into two classes: benign and catastrophic. Seizures associated with benign syndromes are typically well controlled with medications and are often outgrown. In contrast, seizures associated with catastrophic syndromes often persist despite medication or the age of the child, and typically result in more extensive developmental problems.
Here are some of the common seizure syndromes:

1. Benign Epilepsy Syndromes

a. Childhood absence epilepsy (CAE)
- Characterized by recurrence of absence seizures that can occur up to hundred times per day
- Affects children between the ages of 4 – 12 years old
- Most children with CAE outgrow the propensity for seizures by adolescence

b. Benign Rolandic Epilepsy (BRE)
- Affects children between 3 to 16 years old
- Seizures are infrequent and in some cases do not require medication
- Begins with a sensation at the corner of the mouth and drooling, followed by jerking of the mouth that can progress to the rest of that side of the face, and sometimes to that entire side of the body
- Patients are unable to speak for a period of time during and after the seizure.
- Seizures usually occur at night, and usually affecting the facial and pharyngeal muscles

c. Juvenile Myoclonic Epilepsy (JME)
- Begins in late childhood or early adolescence from 8 to 20 years old
- Characterized by mild myoclonic jerks as the individual is going to sleep or waking
- Absence seizures are usually less common in this syndrome
- Patients are often diagnosed when they have the first generalized tonic-clonic seizure later in life when they experience sleep deprivation.
- Alcohol is a contributing factor
- Usually lifelong
- Severity of JME varies from person to person – some will experience full blown tonic-clonic seizures regularly, others will suffer only under sufficient stress from lack of sleep and intoxication

2. Catastrophic Epilepsy Syndromes

a. Infantile Spasms (west syndrome)
- Affects infants from 1 month to 1 year old and very young children
- Typical seizures are characterized by repetitive muscle contractions, sudden flexor and extensor spasms of head, trunk, and extremities
- Spasms occur in clusters, in which one spasm follows another after a brief period of time
- Clusters of infantile spasms typically occur as the child is going to sleep, or soon after waking

b. Doose Syndrome (Myoclonic-Astatic Epilepsy – MAE)
- Affects children between the ages of 1 to 5 years old
- Children with Doose syndrome typically experience mixed seizure types.
- There is no known cause for this syndrome

c. Lennox-Gastaut Syndrome
- Affects children between the ages of 2 to 6 years old.
- Common among children who experienced infantile spasms
- Has no single cause but typically results from some developmental brain disorder or brain injury
- Mixed seizure disorder
- Can have two or more seizure types, one of which is the atonic type of seizure, which causes loss of muscle tone and a sudden collapse to the ground

d. Landau-Kleffner Syndrome
- Rare syndrome characterized by loss of speech due to abnormal electrical activity in the child's brain during sleep
- Child experiences mild seizures and slowly loses ability to understand language and to speak, as well as their interest in communicating
- Causes lifelong language deficits

Sharon C.