Reza Azizimalamiri; Assistant Professor of Paediatric Neurology, Department of Paediatric Neurology, Golestan Medical, Educational, and Research Center , Ahvaz Jundishapur University of Medical Sciences
azizi.ramin@gmail.com
According to the ILAE new classification of epilepsies, West syndrome is among developmental encephalopathies. In these conditions, the epileptic activity itself contributes to severe cognitive and behavioral impairment beyond that expected from the underlying pathology and that these can worsen over time. West syndrome is in continuum with Ohtahara and early myoclonic encephalopathy (EME) as neonatal epileptic encephalopathies. West syndrome could also develop to lennox‑Gastaut syndrome, another developmental encephalopathy of childhood. These epileptic syndromes are severely drug-resistant and have a huge negative impact on the quality of life of children and their families. Almost all the infants with West syndrome have a developmental delay or could regress over time. Therefore, this condition should be considered in every infant with developmental regression.
West syndrome is characterized by epileptic spasms, developmental delay or regression, and an interictal EEG pattern called hypsarrhythmia. Many infants with West syndrome suffer from structural brain disorders or have neurometabolic diseases. Just a minority of these infants is classified as unknown etiology. In some infants who have West syndrome along with the normal gross structure of the brain in their MRI, PET scans of glucose metabolism and SPECT scans of cerebral blood flow often disclose abnormalities. Therefore, normal MRI of the brain is not equal to a normal brain in these infants.
West syndrome is a drug-resistant epileptic syndrome and the response to different treatment modalities is poor. Among the different anti-seizure medications, ACTH and Sabril are the main agents that frequently try in these infants. However, many infants show no acceptable results and frequently need other treatment modalities such as the ketogenic diet. In those infants who had a focal cortical dysplasia, epilepsy surgery could show acceptable results by removing the abnormal cortex and prevent from encephalopathy.
Unfortunately, even after successful control of spasms, these infants would not develop normally and many of them finally will show autistic features. After infancy, many children with West syndrome develop other types of seizure such as atypical absences, tonic, and atonic seizures and will become children with Lennox‑Gastaut syndrome.
Keywords: West syndrome, infancy, drug-resistant
