Use of quantitative EEG in infants with port-wine birthmark to assess for Sturge–Weber brain involvement

Ewen, Joshua B.; Kossoff, Eric H.; Crone, Nathan E.; Lin, Doris; Lakshmanan, Balaji M.; Ferenc, Lisa M.; Comi, Anne M.

doi:10.1016/j.clinph.2009.06.005

Cited by 43 publications

(51 citation statements)

References 15 publications

(17 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…SWS neuroscores were obtained in 27 children (Kelley et al, 2005; Ewen et al, 2009). This score was obtained at clinic visit and is a composite of seizure frequency, hemiparesis, cognition, and vision subscores.…”

Section: Methodsmentioning

confidence: 99%

“…Despite this, EEG remains a valuable tool in SWS, and may be helpful in non-invasive screening for brain involvement in pre-symptomatic infants with a facial port-wine birthmark (Ewen et al, 2009). We hypothesized that the EEG evolution over time first reported by Chao over 50 years ago was accurate and could be replicated in a larger series of children with SWS.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

EEG evolution in Sturge-Weber syndrome

et al. 2014

Self Cite

View full text Add to dashboard Cite

SUMMARY 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.

show abstract

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

EEG evolution in Sturge-Weber syndrome

et al. 2014

Self Cite

View full text Add to dashboard Cite

show abstract

“…25 Ewen et al recently described a quantitative EEG approach that separated a group of nine infants with facial port-wine birthmark into affected and unaffected groups. 26 If successfully applied to larger numbers of infants and EEGs from other facilities, this approach may prove to be a very useful tool for screening at-risk infants to determine who should receive an early MRI with contrast and which parents can be reassured and wait for later imaging.…”

Section: Neurologic Signs and Symptoms In Sturge-weber Syndromementioning

confidence: 99%

Presentation, Diagnosis, Pathophysiology, and Treatment of the Neurological Features of Sturge-Weber Syndrome

2011

Self Cite

View full text Add to dashboard Cite

Background Sturge-Weber syndrome (SWS) is a neurovascular disorder with a capillary malformation of the face (port-wine birthmark), a capillary-venous malformation in the eye, and a capillary-venous malformation in the brain (leptomeningeal angioma). Although SWS is a congenital disorder usually presenting in infancy, occasionally neurologic symptoms first present in adulthood and most affected individuals do survive into adulthood with varying degrees of neurologic impairment including epilepsy, hemiparesis, visual field deficits and cognitive impairments ranging from mild learning disabilities to severe deficits. SWS is a multi-system disorder that requires the neurologist to be aware of the possible endocrine, psychiatric, ophthalmologic, and other medical issues that can arise and impact the neurologic status of these patients. Some of these clinical features have only recently been described. Review Summary This review summarizes the neurologic manifestations of SWS, discusses issues related to the diagnosis of brain involvement, relates major neuroimaging findings, briefly describes the current understanding of pathogenesis, and provides an overview of neurologic treatment strategies. Conclusion Recent clinical research has highlighted several novel and lesser known aspects of this clinical syndrome including endocrine disorders. Functional imaging studies and clinical experience suggests that neurologic progression results primarily from impaired blood flow and that prolonged seizures may contribute to this process. Treatment is largely symptomatic although aggressive efforts to prevent seizures and strokes, in young children especially, may impact outcome.

show abstract

“…Neuroimaging can be done proactively, before symptoms even occur. However, in newborns and young infants, these imaging techniques can often lead to false negative results [6]. For this reason, MRI is not suggested for young infants as it can be falsely reassuring, and imaging all these children (most of whom are normal) would require the sedation of large numbers of normal children.…”

Section: Introductionmentioning

confidence: 99%

“…As mentioned before, early diagnosis of brain involvement is important to either reassure parents or provide a basis for prophylactic treatments. In younger patients, electroencephalography (EEG) is a good option for assessing abnormal brain activity, and identifying patients at risk for future neurologic symptoms [6]. While EEG can also lead to false negatives in young patients who may not have fully developed symptoms, EEG is a useful method because it is non-invasive and can be repeated easily, and safely as often as necessary.…”

Section: Introductionmentioning

confidence: 99%