Electroencephalography (EEG) Evolution in Sturge-Weber Syndrome

SEPTEMBER 26, 2014

Electroencephalography (EEG) Evolution in Sturge-Weber Syndrome

Drs. Kossoff and Comi at the Kennedy Krieger Institute recently published this study in the May 2014 issue of Epilepsy Research (see abstract below).  Eighty-one EEGs from 44 subjects were studied to better understand how their EEG findings related to their neurologic status. There were two main findings:  1. EEG patterns often change over time in patients with Sturge-Weber syndrome 2. The EEG score (rating findings on EEG like the presence of focal slowing, focal sharps and frequent burst of spike and wave) did not correlate with the child’s neurologic function or seizure statusBreaking this down a bit: We know that EEG is often abnormal in individuals with Sturge-Weber syndrome. That is why we often use it as a way to screen for brain involvement in a baby with a port-wine birthmark on forehead. However, the dramatic abnormalities on EEG, sharps and spikes and bursts of spike and wave are more common as the child gets older. We can sometimes use EEG to help make decisions about seizure medications. But this study cautions clinicians and patients to not make too much of sharps or spikes on EEG. By themselves, they do not necessarily mean that seizures or neurologic status are worsening. EEG information has to be understood in the light of how the child is doing other ways. This study further contributes to our understanding of how EEG can best be used as a tool to monitor and treat patients with Sturge-Weber syndrome. We also continue to use and study quantitative EEG which analyses something called power which is a measure of brain function. qEEG compares the affected to the unaffected (or less affected) side. This approach is much more, well, quantitative. It is therefore more sensitive to smaller changes over time and therefore likely to be more useful for tracking responses to treatment and changes over time. We have already shown that it is useful for screening infants with facial port-wine birthmarks (Ewen 2009). Stay tuned. The research world calls this biomarker development. To the patient, this means that we continue the effort to develop non-invasive, safe, and effective ways to monitor treatment response and neurologic progression in Sturge-Weber syndrome.Thanks to all patients and families who have contributed to these studies.

Drs. Kossoff and Comi at the Kennedy Krieger Institute recently published this study in the May 2014 issue of Epilepsy Research (see abstract below). Eighty-one EEGs from 44 subjects were studied to better understand how their EEG findings related to their neurologic status. There were two main findings: 

1. EEG patterns often change over time in patients with Sturge-Weber syndrome
2. The EEG score (rating findings on EEG like the presence of focal slowing, focal sharps and frequent burst of spike and wave) did not correlate with the child’s neurologic function or seizure status

Breaking this down a bit: We know that EEG is often abnormal in individuals with Sturge-Weber syndrome. That is why we often use it as a way to screen for brain involvement in a baby with a port-wine birthmark on forehead. However, the dramatic abnormalities on EEG, sharps and spikes and bursts of spike and wave are more common as the child gets older. We can sometimes use EEG to help make decisions about seizure medications. But this study cautions clinicians and patients to not make too much of sharps or spikes on EEG. By themselves, they do not necessarily mean that seizures or neurologic status are worsening.

EEG information has to be understood in the light of how the child is doing other ways. This study further contributes to our understanding of how EEG can best be used as a tool to monitor and treat patients with Sturge-Weber syndrome.

We also continue to use and study quantitative EEG which analyses something called power which is a measure of brain function. qEEG compares the affected to the unaffected (or less affected) side. This approach is much more, well, quantitative. It is therefore more sensitive to smaller changes over time and therefore likely to be more useful for tracking responses to treatment and changes over time. We have already shown that it is useful for screening infants with facial port-wine birthmarks (Ewen 2009). Stay tuned. The research world calls this biomarker development. To the patient, this means that we continue the effort to develop non-invasive, safe, and effective ways to monitor treatment response and neurologic progression in Sturge-Weber syndrome.

Thanks to all patients and families who have contributed to these studies.

Kossoff EH, Bachur CD, Quain AM, Ewen JB, Comi, AM. (2014). EEG Evolution in Sturge-Weber Syndrome. Epilepsy Research, 108(4), 816-819

Abstract: The EEG in Sturge-Weber syndrome (SWS) was theorized over 50 years ago as changing over time from normality to focal asymmetry to lastly epileptiform. We sought to validate these findings in a larger cohort today. Children with confirmed SWS and routine EEG at our center were evaluated retrospectively. An EEG score (0-3) was created and linked to patient current age, overall neurologic function, and seizure frequency. Eighty-one EEGs from 44 patients with SWS (mean age 2.0 years (range: 0.2-37.9 years)) were evaluated and assigned an EEG score. The mean age for patients with an EEG score of 0-1 (normal or focal slowing) was 3.2 years (SEM 0.6), whereas those with an EEG score of 2-3 (focal sharp waves or frequent spike-wave bursts) was 8.7 years (SEM 1.7) (p=0.006). There was no correlation between the EEG score and either the SWS overall neuroscore or seizure subscore (measuring frequency). The EEG in patients with SWS does appear to evolve over time, becoming more abnormal with more frequent epileptiform activity, as suspected in smaller studies decades ago. This progressive change, however, did not correlate with the child's neurologic function or seizure frequency.

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