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West syndrome: a university hospital based study from Oman
1Division of Pediatric Neurology, Department of Child Health, Sultan Qaboos University Hospital, P.B. No. 38, Al Khod 123, Oman. rkoul@omantel.net.om
Insights
West syndrome, a severe epilepsy, often presents with developmental delay and hypsarrhythmia. Treatment with sodium valproate and vigabatrin showed limited seizure control and developmental improvements in children.
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.
Abstract:
Forty-four children (20 male: 24 female) with West syndrome (infantile spasms, mental retardation/regression and hypsarrhythmia) diagnosed at Sultan Qaboos University Hospital (Pediatric Neurology Division of the Department of Child Health) are reported, with thirty-four (77.3%) children constituting the symptomatic group. All children were followed up for an initial 1 year at this hospital. Thirty-seven cases (84%) still continue their follow-up with us. The age of onset ranged from 1 to 14 months (mean, 6.0 months). Developmental delay before the onset of infantile spasms was noted in 29 (65.9%) children. Brain computed tomography was abnormal in 29 (65.9%). Sodium valproate and vigabatrin were the most often used drugs, though other antiepileptic drugs were also used. Nine (24.5%) children achieved good seizure control, out of which five have normal development. Only one child could be weaned off antiepileptic drugs completely. There was one death in the whole series, related to aspiration pneumonia.