Evolution of Electroclinical Characteristics of West Syndrome: A Hospital Based Retrospective Study

Introduction: West syndrome (WS) is an epileptic encephalopathy with onset between 4 and 8 months of age. Long-term prognosis of epilepsy and developmental outcome in WS remains under-explored. This study explains evolution of electroclinical characteristics in WS.

Objective: (1) To compare electroclinical characteristics between cryptogenic and symptomatic WS; (2) to compare effectiveness of treatment modalities with respect to clinical and developmental outcome; (3) to assess evolution of EEG pattern in WS; (4) to study clinical outcome of symptomatic WS with respect to imaging findings.

Methods: Electro-clinico-radiological data of 76 children with WS were collected retrospectively by reviewing case records and followed-up after 1 year for clinical and developmental outcomes.

Results: Among 76 children enrolled, 31 (40.8%) had cryptogenic and 45 (59.2%) had symptomatic etiology. Children with symptomatic WS (p = 0.037) with gliosis on imaging (p = 0.05) were more likely to have other seizure types before onset of spasms and exhibit delay or regression in milestones (p = 0.017). Of 71 patients who had modified hypsarrythmia pattern, those with other seizure types had multifocal spikes (p = 0.021). There was negative correlation between time to diagnosis and reduction in Engel scores (r = —0.32, p = 0.01). There was significant reduction in Engel scores with ACTH, irrespective of etiology (p < 0.001 in cryptogenic and symptomatic) or dose (p = 0.021 in high [> 20 IU] and p < 0.001 in low dose groups). Those with asymmetric hypsarrythmia or multifocal spikes showed better clinical improvement (p = 0.004). Those who failed to achieve seizure remission most likely exhibited developmental delay or regression (p = 0.019).

Conclusion: Early diagnosis and appropriate treatment with ACTH and antiepileptic medications is the key toward improved outcome in WS, irrespective of etiology.