Infantile Epileptic Spasms Syndrome (IESS)
Incidence:
1 in 3,000 children. Onset in the first year of life, mainly 4-8 months of age.
Causes of IESS (70%):
- Congenital malformation of the brain (Cortical dysplasia, lissencephaly, holoprosencephaly)
- Birth asphyxia
- Meningitis/Encephalitis
- Mutation in a gene & chromosomal abnormalities. Common in Down syndrome.
- Neurocutaneous syndromes mainly tuberous sclerosis.
- Inborn errors of metabolism (Phenylketonuria, congenital infections affecting the CNS such as toxoplasmosis, syphilis, cytomegalovirus, and Zika virus)
Idiopathic (30%).
- West syndrome: The triad of epileptic spasms, arrest of psychomotor development, and EEG pattern known as hypsarrhythmia.
Characteristics of the Seizures
- Brief axial contractions
- Usually bilateral, may be asymmetrical
- Typically flexor, may be extensor
- Usually in clusters, less likely random
- Typically on awakening, or when drowsy
- EEG shows hypsarrhythmia (In West syndrome): - Multifocal spikes - High voltage, chaotic background.
Treatment:
Respond to prednisolone/ACTH IM/Vigabatrin
Hypsarrhythmia. This is a pattern of disorganized background activity and high-voltage spikes and slow waves.
