Epilepsy Research UK - funding independent research into epilepsy since 1991
Epilepsy Research UK Logo
 

 

Title Bullet Childhood epilepsy syndromes
 

There are many different types of childhood epilepsy syndrome, five of which are outlined below. When diagnosing an epilepsy syndrome, factors such as the type of seizure the child is having, their age at seizure onset, their gender and whether or not they have learning difficulties are very important. Once a doctor has diagnosed a particular syndrome, he/she will be able to consider the best course of treatment and the likely prognosis.

Rolandic epilepsy
Rolandic epilepsy is the most common of the childhood epilepsy syndromes, affecting approximately one in 4,800 children under 15 years. It is characterised by partial seizures that usually occur during sleep or just before wakening, and involve the face, mouth and speech organs (although in some cases seizures may be more generalised).

Rolandic epilepsy normally occurs between the ages of three and 15 years (most commonly between six and eight years), but the cause, although suspected to be genetic, is not fully established. Most children grow out of the condition when they reach adulthood, and because seizures occur at night and learning is not usually impaired, neurologists often refrain from treating it with anti-epileptic drugs. It is often referred to as 'benign'.

Epilepsy Research UK is currently funding a study at Bristol University, using a restricted grant from the Waterloo Foundation. This is looking at the link between sleep disturbance and learning difficulties, and whether treatment of Rolandic epilepsy as standard practice would improve the learning outcomes of affected children.

West's syndrome (infantile spasms)
West's syndrome is a disorder of the brain that occurs in approximately one in every 5,000 babies. It is characterised by infantile spasms (a form of epilepsy that usually develops before the age of one, often at about six months), a specific EEG pattern, developmental regression and, often, learning/physical disabilities. Many of the conditions that cause West's syndrome are genetically inherited, but this is not always the case; so it is very important to establish the exact cause in each child.

Treatments for West's syndrome include the anti-epileptic drugs and oral steroids. Children's responses to these medications a highly variable, however, and cannot be predicted. In cases of drug resistance, epilepsy surgery may be considered.

Rett syndrome
Rett syndrome is a neurodevelopmenal disorder that affects girls almost exclusively. It is characterised by normal early growth and development, followed by a slowing of development, loss of purposeful use of the hands, distinctive hand movements, slowed brain and head growth, problems with walking, seizures, and intellectual disability.

It is estimated that one in 10,000 to 15,000 female babies will be affected by Rett Syndrome, sometimes as early as six months of age. Nearly all cases genetic, but it is thought that 99% of genetic cases are due to spontaneous mutations, not defects inherited from the parents.

There is no cure for Rett Syndrome and most treatments are symptomatic, for example anti-epileptic drugs for seizures and other medications for breathing/movement irregularities.

Angelman syndrome
Angelman syndrome is a genetic neurological disorder that occurs in approximately one in every 25,000 children. It is characterized by intellectual and developmental delay, sleep disturbance, seizures, jerky movements (especially hand-flapping), frequent laughter or smiling, and usually a happy demeanor. It is usually diagnosed between the ages of three and seven, when the clinical symptoms become apparent; but developmental delay may begin as early as six months.

There is no specific therapy for Angelman syndrome, but treatment for seizures is usually needed. Physical and occupational therapies, communication therapy, and behavioral therapies are important in allowing individuals with Angelman syndrome to reach their maximum developmental potential.

Lennox-Gastaut syndrome
Lennox-Gastaut syndrome is a 'difficult-to-treat' form of childhood-onset epilepsy that affects approximately one in 50,000 children, and usually appears between the second and sixth years of life. It is characterised by frequent seizures of different types and is often accompanied by mental retardation, psychological and behavioral problems. In up to a third of cases, no cause can be found.

Lennox-Gastaut is notoriously resistant to anti-epileptic medication and often multiple drugs. in high doses must be prescribed. If there is still no improvement, alternative treatments such as the ketogenic diet or epilepsy surgery may be considered.

 
 
 
 
 
 
 
up arrow
 
 

Copyright © Epilepsy Research UK 2011 / Website by Pipedream

Information about epilepsy | Support epilepsy research | About research into epilepsy
About Epilepsy Research UK | Epilepsy research news | Researchers and scientists