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West syndrome

Background

West syndrome is the term used to describe a type of epilepsy which most typically starts in the first year of life, between four and 8 months of age. It may, however, start younger than this.

Credits

Medical text written November 2002 by Dr J Livingston, Consultant Paediatric Neurologist, Leeds General Infirmary, Leeds, UK. Last updated October 2015 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?

The hallmark of West syndrome is the occurrence of a particular type of epileptic seizure called a spasm. Spasms typically produce a sudden jerk of the body followed by stiffening of the limbs. Different types of spasms may occur, but most typically a child will suddenly bend forward with elevation of the arms or legs. These attacks usually occur in runs or clusters when one spasm occurs after another at five to 15 second intervals for a period of several minutes. These episodes may occur several times per day. Very commonly, when a child starts to have spasms, there is change in their behaviour and they stop vocalising and making visual contact.  This commonly persists.

What are the causes?

West syndrome is not one condition but is a symptom of many different brain disorders. A cause can be found in most children, but in a small proportion no obvious cause can be identified. Sometimes West syndrome occurs in children with severe abnormalities of the brain such as congenital infections, severe brain injury due to birth asphyxia (lack of oxygen) or severe malformations of the brain. Sometimes very rare genetic or metabolic diseases cause West syndrome. One of the most common causes of West syndrome is tuberous sclerosis (see entry Tuberous Sclerosis Complex). Chromosome abnormalities may also cause West syndrome. It is therefore very important when a diagnosis of West syndrome is made that an extensive search is carried out to find the underlying cause.

How is it diagnosed?

The diagnosis of West syndrome is based on the occurrence of infantile spasms and the presence of a very abnormal EEG (brain wave recording). The most typical EEG abnormality is called 'hypsarrhythmia'; however, other abnormal patterns may occur.

How is it treated?

The most common treatments for West syndrome are vigabatrin (an anti-epileptic drug) or corticosteroids (prednisolone, hydrocortisone, corticotrophin, or adrenocorticotropin [ACTH]). In many children the spasms will stop with appropriate treatment; however, after a period of time they will often relapse and require a change of treatment. Some would now suggest treatment with both drugs.

Inheritance patterns and prenatal diagnosis

Inheritance patterns 
This depends on the underlying cause and genetic advice should be sought.

Prenatal diagnosis 
None.

Is there support?

West Syndrome Support Group

Tel: 01252 654 057
Email: kimdenise44@gmail.com

The Group is run by parents and offers support and a sympathetic ear to families of children affected by West Syndrome.

Group details last updated February 2016.

UK Infantile Spasms Trust

Email: via website
www.ukinfantilespasmstrust.org

The Trust is a Registered Charity no. 1172167. They provide information and support to children, parents and carers of those affected by Infantile Spasms or West Syndrome. They also offer an active Facebook group. They aim to promote research and raise awareness of the conditions.

Group details added April 2017.

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