Page 67 - ILAE_Lectures_2015

Table 1. Main causes of West syndrome. Most can also cause the Lennox-Gastaut syndrome.

Prenatal disorders Disease category Examples
Neurocutaneous syndromes Tuberous sclerosis
Chromosomal and genetic disorders Sturge-Weber syndrome
Incontinentia pigmenti
Malformations of cortical Down syndrome
development Miller-Dieker syndrome
Fragile X syndrome
Hypoxic-ischaemic insults X-linked infantile spasms
Congenital infections Aicardi syndrome
Metabolic disorders Lissencephaly
Pachygyria
Polymicrogyria
Laminar heterotopias
Hemimegalencephaly
Schizencephaly
Holoprosencephaly

Cytomegalovirus
Rubella
Toxoplasma
Pyridoxine dependency
Amino and organic
acidopathies
Mitochondrial disorders

Perinatal disorders Hypoxic ischaemic insults
Hypoglycaemic brain damage
Severe infections Meningitis
Encephalitis

Birth trauma
Intracranial haemorrhage

Postnatal disorders Severe infections Meningitis
Encephalitis
Trauma
Intracranial haemorrhage Early onset polio and
Neurodegenerative disease leukodystrophies
Theophylline
Drugs

West syndrome is usually easily recognised but can be confused with a number of epileptic
and non-epileptic disorders, including: benign (non-epileptic) myoclonus of infancy; benign
neonatal sleep myoclonus; Sandifer’s syndrome; and colic.

There are many treatment strategies for West syndrome. Conventional AEDs may have a role
and in France high-dose sodium valproate is often used first line. Pyridoxine is popular,
particularly in Japan. However, in most countries the choice of initial treatment is usually
between vigabatrin and steroid treatment. With regard to steroids, preparations used most
often are natural and synthetic ACTH given intramuscularly and oral prednisolone.
Vigabatrin is particularly effective for spasms due to tuberous sclerosis. A recent UK