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Epilepsy syndromes in Childhood

Background

The epilepsies are a group of conditions in which people are prone to have attacks (epileptic seizures or fits). A seizure is caused by a burst of excess electrical activity in the brain, causing a temporary disruption in the normal message passing between brain cells. This disruption stops the normal brain activity and produces changes in behaviour and movement.

The epilepsy syndromes of infancy and childhood broadly separate into benign and more severe. The benign conditions usually occur in an otherwise normal child and are of one type of attack, normally easily treated and tending to show natural remission over time. Severe conditions are harder to treat and have a larger impact on the child's and their family's quality of life.

Benign syndromes, including febrile seizures (convulsions brought on by a fever in infants or small children), and benign epilepsy with centro-temporal spikes (named because of a specific pattern of brain waves seen on an electroencephalogram (EEG)) quite often don't need treatment.

Credits

Medical text written December 2010 by Professor Brian Neville, Emeritus Professor of Paediatric Neurology, UCL Institute of Child Health and Great Ormond Street Hospital, London, UK.

What are the symptoms?

Seizures are of many types:

  • tonic (stiffening)
  • clonic (jerking)
  • combinations of both tonic and clonic
  • loss of awareness
  • myoclonus (sudden jerks of the whole body or limb)
  • abnormal feelings of fear, smell, taste, vision and/or feeling in a part of the body with or without impairment of consciousness, all pointing to a focal onset (specific part of the brain from which the seizure originates)
  • automatic movements (such as fiddling with clothes or objects, mumbling or making chewing movements).

All types of epilepsy have a small risk of sudden unexplained death (known as sudden unexpected death in epilepsy (SUDEP)), which is difficult to anticipate but has brought about the development of epilepsy networks and guidelines for effective diagnosis, investigation and treatment.

The more severe syndromes include a number of conditions that may be difficult to treat and which cause severe regression (loss of acquired skills) relating to learning, social functioning and behaviour.

Thus, learning impairments (see entry Learning Disability), autism spectrum disorder (see entry Autism Spectrum disorders, including Asperger syndrome), attention deficit hyperactivity disorder and other behavioural disorders are very common in children with intractable seizures (seizures that are difficult to control).

The regression in development, which is particularly caused by early-onset epilepsy is known as an epileptic encephalopathy. This is particularly related to sub-clinical epileptic activity (activity that is not obvious) rather than the rate of obvious seizures.

What are the causes?

Epilepsy in childhood has a wide range of causes, including a large number of specific syndromes, many of which have a genetic basis. Many illnesses involving the brain, including infection, trauma and metabolic abnormalities can cause epilepsy.

Epileptic seizures are, therefore, a symptom of very many conditions that involve the brain and these often require investigation in their own right by a paediatrician/paediatric neurologist.

Specific syndromes such as Sturge-Weber syndrome and tuberous sclerosis complex may give rise to epilepsy.

How is it treated?

Many children with epilepsy don't fit into one syndrome and management is along the lines of the closest epilepsy type. Children with epilepsy may be offered an anti-epilepsy drug (AED), which can prevent seizures in some cases. Examples of AEDs commonly used in children include sodium valproate, carbamazepine, lamotrigine and levetiracetam. Doctors need to choose AEDs carefully as they may be effective in only one type or a few types of epilepsy (narrow spectrum), whilst others treat a much wider range of epilepsies (broad spectrum). Guidelines on the use of AEDs are available from the National Institute of Clinical Excellence (NICE). Although 60 to 70 per cent of seizures stop with the first one of two AEDS used, if two AEDs fail to control a child's seizures then chance of freedom from seizures is small. Sometimes, drug treatments are not those normally used in epilepsy, for example corticosteroids are used to treat types of epilepsy such as West syndrome and Landau-Kleffner syndrome.

Surgery is being increasingly used as early as possible for those with a clearly identifiable and removable source of the seizures in the brain in order to minimise secondary impairments. Surgery is only possible in a small minority.

The additional impairments associated with epilepsy in childhood require diagnosis and treatment in their own right. They are often much more disabling than the seizures and have educational implications. In a recent study, about half of the children with epilepsy in childhood had problems with either behaviour, educational progress or both. Most children with epilepsy (about 75 per cent) become seizure free on AEDs, but in the rest the above problems are common.

Inheritance patterns and prenatal diagnosis

Inheritance patterns
The genetics of epilepsy are a mixture of specific genetic and non-genetic conditions and those without such clear diagnosis in which a small increased susceptibility for siblings is seen.

Prenatal diagnosis
This is only available for a limited number of genetically determined conditions.

Is there support?

Epilepsy Action

Helpline: 0808 800 5050
Email: helpline@epilepsy.org.uk
www.epilepsy.org.uk

The Organisation is a Registered Charity in England and Wales No. 234343. It offers information and support to anyone affected by epilepsy. The Organisation has a network of groups across the UK.

Group details last updated January 2016.

Epilepsy Scotland

Helpline: 0808 800 2200
Email: helpline@epilepsyscotland.org.uk
www.epilepsyscotland.org.uk

The Organisation is a Registered Charity in Scotland No. SC 000067. It provides information and support to people of all ages who are affected by epilepsy. The Organisation has specific support services for children and families including support groups, youth workshops and social work assessments.

Group details last updated January 2016.

Epilepsy Society

Helpline: 01494 601 400
Email: via website
www.epilepsysociety.org.uk

The Society is a Registered Charity in England and Wales No. 206186. It provides information and support to anyone in the UK who is affected by epilepsy.  

Group details last updated January 2016.

Epilepsy Wales

Helpline: 0800 228 9016
http://epilepsy.wales

Epilepsy Wales is a Registered Charity in England and Wales No. 1059067, established in 1996. It aims to help people with epilepsy in Wales; their families and carers by offering a bi-lingual service wherever possible; support groups and field workers throughout Wales.

Group details last updated October 2015.

Young Epilepsy

Helpline: 01342 831 342
Email: helpline@youngepilepsy.org.uk
www.youngepilepsy.org.uk

The Organisation is a Registered Charity in England & Wales No. 311877. It provides information and support for young people aged 5-25 with epilepsy, autism and other neurological conditions, and their parents. The Organisation offers specialist services including a school, college and residential services.

Group details last updated September 2014.

Talk to other families about your child's condition

Visit our online Epilepsy syndromes in Childhood group

Visit our online advice and support or read all about diagnosis.


You can also call us if you need information or advice on any aspect of caring for a disabled child. Monday to Friday, 9.30am to 5pm.


0808 808 3555 or helpline@contact.org.uk