West syndrome: a university hospital based study from Oman

Area of Science:

• Pediatric Neurology
• Clinical Pediatrics
• Epileptology

Background:

• West syndrome, characterized by infantile spasms, developmental regression, and hypsarrhythmia, poses significant challenges in pediatric neurology.
• Early diagnosis and management are crucial for improving outcomes in affected children.

Purpose of the Study:

• To report on the clinical characteristics, treatment, and outcomes of children diagnosed with West syndrome at Sultan Qaboos University Hospital.
• To evaluate the efficacy of commonly used antiepileptic drugs in this cohort.

Main Methods:

• Retrospective analysis of 44 children diagnosed with West syndrome.
• Data collection included age of onset, clinical presentation, neuroimaging findings, treatment regimens, and follow-up outcomes.
• Assessment of seizure control and developmental status post-treatment.

Main Results:

• The majority of children (77.3%) had symptomatic West syndrome.
• Developmental delay was present before spasms in 65.9% of cases, with abnormal brain CT scans in the same proportion.
• Good seizure control was achieved in 24.5% of children, with five showing normal development; only one child was weaned off medication.
• One death occurred due to aspiration pneumonia.

Conclusions:

• West syndrome in this cohort was frequently symptomatic, often preceded by developmental delay, and associated with significant neuroimaging abnormalities.
• Current antiepileptic drug regimens, including sodium valproate and vigabatrin, offer limited efficacy for seizure control and developmental recovery.
• Further research into novel therapeutic strategies is warranted to improve the prognosis for children with West syndrome.