Epilepsy
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Common Epilepsy Syndromes
A syndrome is a disorder consisting of a group of signs and symptoms that commonly occur together. Identification of an epileptic syndrome may help the neurologist to determine genetic risk as some syndromes are associated with specific genotypes (i.e. the genetic constitution of an organism). The following are examples of common epilepsy syndromes seen in childhood.
Lennox Gestaut is defined by a combination different types of seizures, mental retardation and psychomotor slowing. This syndrome may develop over the course of months or sometimes within weeks. The prognosis for complete seizure control is poor. These children can often go into status epilepticus which is a seizure or a series of seizures that last more than 30 minutes with no recovery of consciousness during that period of time. Children with this disorder often have severe expressive and receptive language abilities as well as problems in the regulation of attention, behavior (e.g. hyperactivity) and emotion (e.g. irritability). Some children become psychotic. There may be a progressive deterioration in cognitive functioning over time.
Benign Rolandic epilepsy is a syndrome of genetic basis consisting of generalized seizures that occur at night. The disorder begins in childhood with the age of onset usually between 3 and 13 years of age. Boys are affected more than girls. The neurological and neuropsychological evaluation are usually normal. This syndrome responds very well to medication. Some children with this syndrome may not need medication. The seizures can stop by age fifteen.
Landau-Kleffner Syndrome (LKS) is an epilepsy syndrome the cause of which is unknown. Onset is usually between ages 2 and 8. EEG abnormalities are often found during sleep originating in the temporal lobes of the brain resulting in expressive or receptive language dysfunction. Language comprehension is very often affected and can be mistaken for deafness, autism or a developmental language disorder. Children with LKS may be unable to comprehend their own name or recognize common sounds from the environment (e.g. fire engine, doorbell). Autistic type of behavior (e.g. poor eye contact, repetitive play, aggression) and visual disturbances may also be observed. LKS is difficult to fully control. Anti-epileptic seizure medications and/or steroids may be used.
Juvenile myoclonic epilepsy (JME) is a syndrome that often begins around the time of puberty. A positive family history is common. Typical features include myoclonic jerks of the upper extremities, neck and shoulders and/or tonic-clonic seizures without loss of consciousness. The seizures often occur after awakening or during early morning sleep. JME may be worsened by sleep deprivation. The neurological and neuropsychological evaluation are usually normal.