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West syndrome in tuberous sclerosis complex
A M Husain1, C M Foley, A Legido
1Department of Medicine, Division of Neurology, Duke University and Neurodiagnostic Center, Veterans Affairs Medical Center, Durham, North Carolina 27710, USA.
Insights
West syndrome in children with tuberous sclerosis complex can improve, contrary to previous reports. EEG sleep patterns showed promise in predicting seizure control, while steroid therapy duration did not impact outcomes.
Area of Science:
- Pediatric Neurology
- Epileptology
- Genetics
Background:
- West syndrome is common in tuberous sclerosis complex (TSC), often linked to poor cognitive and seizure prognosis.
- Previous studies suggest high mortality and intractable epilepsy in these children.
Purpose of the Study:
- To investigate epilepsy outcomes in children with TSC and West syndrome.
- To identify factors (EEG, MRI, steroid therapy) differentiating seizure improvement from intractable epilepsy.
Main Methods:
- Retrospective analysis of 17 patients with TSC and West syndrome.
- Collected serial EEG, MRI, and treatment data over at least 12 months.
- Compared data between groups with improved versus intractable seizures.
Main Results:
- Seizure control improved in 10 patients and worsened in seven; no mortality observed.
- No significant differences found in EEG background, MRI findings, or steroid treatment duration.
- EEG sleep patterns approached statistical significance as an indicator of seizure control (P = 0.06).
Conclusions:
- Findings challenge reports of uniformly poor epilepsy outcomes and high mortality in this population.
- EEG sleep patterns may be a valuable indicator for seizure control in West syndrome with TSC.
- Steroid therapy duration did not influence long-term seizure control.
Abstract:
West syndrome occurs commonly in children with tuberous sclerosis complex and is associated with a grave prognosis for cognitive and seizure outcomes. We sought to determine the epilepsy outcome of children with tuberous sclerosis complex and West syndrome and whether EEG, MRI, or steroid therapy duration were different in those whose epilepsy improved compared with those with intractable seizures. Seventeen patients with tuberous sclerosis complex and West syndrome were identified. For each patient, two sets of clinical evaluations, EEG and MRI data, and treatment information separated by at least 12 months were obtained. The patients were divided into two seizure outcome groups. EEG, MRI, and treatment data were compared between the groups. The intellectual deficiency was either severe (76%) or moderate (24%). Seizure control improved in 10 and worsened in seven, without mortality (follow-up range = 12-216 months). No significant differences in EEG background, MRI findings, or steroid treatment duration were evident between the groups. The difference in EEG-sleep approached statistical significance (P = 0.06). Our findings did not confirm reports of high mortality and poor epilepsy outcome in intellectually deficient children with West syndrome and tuberous sclerosis complex. EEG sleep was the best indicator of seizure control and approached statistical significance. The duration of steroid therapy had no influence on seizure control.