Syndromes


After epilepsy has been diagnosed in a person, it is important for a doctor to determine whether the patient has any epilepsy syndromes. Classifying epilepsy into different syndromes takes a number of factors into account including: the types of seizures, typical EEG recordings and behaviour during seizures. The identity of an epilepsy syndrome can help to determine genetic risks (as certain syndromes are associated with specific genotypes). From this prognosis the doctor will be able to advise on appropriate therapies and treatment. It is important to note that the occurrence of a syndrome with epilepsy is relatively rare. Outlined below are a few of the more prevalent syndromes people with epilepsy face.

Benign Rolandic Epilepsy

Benign Rolandic epilepsy (also known as benign partial epilepsy of childhood) accounts for more than one-third of all cases of epilepsy that begin in middle childhood, accounting for 16 percent of those beginning before age 15. There is a family history in 18 percent of cases and the condition is probably genetically determined. Rolandic epilepsy is a common type of benign partial epilepsy. Seizures start as simple partial, usually beginning in the face. There may be drooling and temporary inability to speak, although consciousness is preserved. The seizures then may generalize to tonic-clonic convulsions. Most of the seizures are nocturnal (occurring during sleep). Neurological and other functioning is usually normal, while the EEG shows a dramatic focal spike most often in the centrotemporal regions of the brain. Most children are seizure free five years after onset; by age 14, 95 percent will have undergone permanent remission.

Landau-Kleffner Syndrome

Landau-Kleffner syndrome, a rare disorder, causes children to develop the inability to speak. They have trouble understanding speech and may seem not to hear or understand what is said to them. Many of these children will also have seizures that vary in type. Speech in children with the syndrome slowly declines over time. There are epilepsy-related abnormalities on the EEG, even though some of the children may not have seizures. Treatment with standard anti-epileptic drugs is ineffective. However, treatment with steroids has been tried with some success. Multiple subpial transection, (a form of surgery designed to spare the speech areas), has also been tried. Language for many of these children will improve slowly over time, but may not return to a normal level for their age.

Lennox-Gastaut Syndrome

Lennox-Gastaut syndrome (also known as myoclonic-astatic epilepsy) is a combination of seizures usually including atypical absence seizures (starting with automatic behavior without conscious control); tonic seizures (stiffening), atonic or astatic seizures (drop attacks); intellectual disabilities; distinct EEG tendings. These children are often developmentally normal when the syndrome begins, but then lose skills, sometimes dramatically, in association with uncontrolled seizures. By age 6 most children with Lennox-Gastaut syndrome have some degree of intellectual disability. Children with Lennox-Gastaut syndrome typically have more than one type of seizure. The atonic-astatic (drop attack) seizures are most troubling because of the injuries caused by repeated falls. As a direct result many children wear protective helmets. The tonic (stiffening) seizures are most common during sleep, including nap time, whereas generalized tonic-clonic seizures (convulsions) occur most often upon awakening.

Rasmussen’s Syndrome

Rasmussen’s syndrome, also known as Rasmussen’s encephalitis, begins in childhood and produces a slow deterioration of one whole side (hemisphere) of the brain with loss of function on the opposite side of the body. An autoimmune response to a viral infection has been suggested as a possible cause. Various types of treatment have been tried, including surgical removal of the affected side of the brain. In children, the remaining hemisphere may compensate for functions lost, but weakness on the affected side will remain. The condition typically starts with seizures, with weakness appearing later in the course of the disorder. Simple partial seizures affecting movement are the most common form.

Reprinted in part from The Epilepsy Foundation







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