Clinical profile of children with West syndrome: A retrospective chart review

This study was intended to document the clinical profile and treatment outcome of West syndrome in children attending a tertiary care centre in Northern India.

Data were collected by a retrospective chart review of children diagnosed with West syndrome between January 2017 to January 2018. Information was recorded pertaining to the age at onset and presentation, etiology, and associated co-morbidities; results of electroencephalography (EEG) and neuroimaging; treatment given; and final outcome. The following drugs were used for treatment: ACTH (n = 7), prednisolone (n = 17), vigabatrin (n = 25), sodium valproate (n = 28), clonazepam (n = 30), and levetiracetam (n = 13) and modified Atkins diet (n = 7). The response was categorized as spasm cessation, partial improvement (>50% improvement), or no improvement.

Records of 30 children (21 boys) were analyzed. The median (IQR) age at onset was 4 (3, 6.5) months. The median (IQR) lag time to treatment was 5 (2,14) months. Eight (26%) were premature, 2 (7%) were small for gestational age, birth asphyxia in 56%, neonatal encephalopathy in 62%. EEG findings were hypsarrhythmia in 13 (43.3%) children and modified hypsarrhythmia in 9 (30%) children. MRI finding was periventricular leukomalacia (54.1%), cystic encephalomalacia (13.8%), normal MRI (20.7%) and one had arrested hydrocephalus. There was no improvement with valproate (93%), clonazepam (89%), levetiracetam (78%). Cessation of spasm was achieved with vigabatrin (28%), prednisolone (38.2%), ACTH (42.8%). Hypsarrhythmia resolved with improvement in of background and other epileptiform abnormalities in 17 children.

The present research highlights favourable response of West syndrome to oral steroids, vigabatrin and ACTH with limited role of conventional antiepileptic drugs like sodium valporate, levetiracetam and clonazepam. Primary care physician plays a vital role in early recognition and treatment of epileptic spasm.