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Partial seizures in West syndrome
Y Ohtsuka1, I Murashima, T Asano
1Department of Child Neurology, Okayama University Medical School, Japan.
Insights
Partial seizures (PS) occur in over a third of children with West syndrome (WS), often associated with less favorable seizure prognosis and specific clinical features. Understanding PS patterns is crucial for assessing underlying cortical lesions in WS.
Area of Science:
- Pediatric Neurology
- Epileptology
Background:
- West syndrome (WS) is a severe infant epilepsy characterized by infantile spasms, hypsarrhythmia, and developmental delay.
- Partial seizures (PS) are less commonly studied in the context of WS, particularly in older children.
Purpose of the Study:
- To investigate the occurrence and characteristics of partial seizures (PS) in children diagnosed with West syndrome (WS).
- To elucidate the electroclinical and radiologic features associated with PS in WS patients.
Main Methods:
- A cohort of 92 children with WS, older than 3 years at follow-up, were analyzed.
- Electroclinical and radiologic data were collected and assessed.
Main Results:
- Partial seizures (PS) were observed in 39.1% of children with WS.
- Children with PS showed a higher incidence of asymmetric spasms, hemiparesis, and asymmetric hypsarrhythmia, with a less favorable seizure prognosis.
- The timing and characteristics of PS varied, with early-onset PS often showing high frequency and variability, while later-onset PS were more stereotyped and originated from frontal regions.
Conclusions:
- Cortical lesions may contribute to both spasms and PS in some WS patients, while others exhibit more diffuse cortical hyperexcitability.
- Focal discharges in WS require cautious interpretation as they can fluctuate and may not always indicate a surgically accessible lesion.
- Stable focal discharges correlating with consistent clinical patterns suggest a greater likelihood of an underlying cortical lesion.
Purpose:
To study the occurrence of partial seizures (PS) and elucidate the characteristics of patients with West syndrome (WS) with PS.
Methods:
We investigated the electroclinical and radiologic features in 92 children with WS who were older than 3 years at follow-up.
Results:
Thirty-six (39.1%) children had PS at some time during the course of the disease. They had a significantly high incidence of asymmetric spasms, hemiparesis, and asymmetric hypsarrhythmia. Their seizure prognosis was significantly less favorable. PS appeared only before spasms (group A, six children), concomitant with spasms (group B, 18 children), and only after spasms ceased (group C, 12 children). Five children in group B had PS after spasms stopped. PS in group A and during the period of active spasms in group B showed high seizure frequency and variability of both seizures and EEG manifestations. The PS and EEG epileptiform discharges were mainly in parietal-posterior temporal-occipital, and central regions. Late PSs in group B and PSs in group C were characterized by stereotyped manifestations and relatively low seizure frequency. These PSs originated mostly from frontal regions.
Conclusions:
In some children with WS, cortical lesions can induce both PS and spasms in series, whereas in others, both types of seizures occur with abnormally increased excitability throughout the cortex rather than as the result of a single cortical lesion. These findings suggest caution in interpreting focal discharges, which may fluctuate and are not necessarily indicative of a surgically accessible lesion. When the discharges are stable and correlate with an equally stable clinical pattern, the likelihood of an underlying cortical lesion is greater.