Quantitative EEG improves prediction of Sturge-Weber syndrome in infants with port-wine birthmark

•Quantitative EEG helps predict which infants with facial port-wine birthmark are most likely to develop Sturge-Weber syndrome.•Risk prediction is critical for emerging presymptomatic treatment of infants with port-wine birthmark.•Long-term collection of qEEG, clinical EEG, history and neurological...

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Published inClinical neurophysiology Vol. 132; no. 10; pp. 2440 - 2446
Main Authors Gill, Ryan E., Tang, Bohao, Smegal, Lindsay, Adamek, Jack H., McAuliffe, Danielle, Lakshmanan, Balaji M., Srivastava, Siddharth, Quain, Angela M., Sebold, Alison J., Lin, Doris D.M., Kossoff, Eric H., Caffo, Brian, Comi, Anne M., Ewen, Joshua B.
Format Journal Article
LanguageEnglish
Published Elsevier B.V 01.10.2021
Subjects
Diagnostic biomarker
Ischemia
Port-wine birthmark
Predictive model
Prognostic biomarker
Quantitative EEG
Sturge-Weber syndrome
qEEG
EEG
LR
KKI-SWSC
Predictive model
Quantitative EEG
STARD
LOOCV
Sturge-Weber syndrome
SWS
AUC
Diagnostic biomarker
SWS-NRS
Ischemia
Port-wine birthmark
NPV
PPV
PWB
Prognostic biomarker
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Summary:•Quantitative EEG helps predict which infants with facial port-wine birthmark are most likely to develop Sturge-Weber syndrome.•Risk prediction is critical for emerging presymptomatic treatment of infants with port-wine birthmark.•Long-term collection of qEEG, clinical EEG, history and neurological exam data allow initial development of a predictive model. Port-wine birthmark (PWB) is a common occurrence in the newborn, and general pediatricians, dermatologists, and ophthalmologists are often called on to make an assessment of risk for Sturge-Weber syndrome (SWS) due to workforce shortages in pediatric neurologists and MRI’s low sensitivity for SWS brain involvement in infants. We therefore aimed to develop a quantitative EEG (qEEG) approach to safely screen young infants with PWB for SWS risk and optimal timing of diagnostic MRI. Forty-eight infants (prior to first birthday) underwent EEG recording. Signal processing methods compared voltage between left and right sides using a previously defined pipeline and diagnostic threshold. In this test sample, we compared sensitivity/specificity of the qEEG metric against MRI performed after the first birthday. We also used likelihood ratio testing to determine whether qEEG adds incremental information beyond topographical extent of PWB, another risk marker of brain involvement. qEEG helped predict SWS risk in the first year of life (p = 0.031), with a sensitivity of 50% and a specificity of 81%. It added about 40% incremental information beyond PWB extent alone (p = 0.042). qEEG adds information to risk prediction in infants with facial PWB. qEEG can be used to help determine whether to obtain an MRI in the first year of life. The data collected can assist in developing a predictive model risk calculator that incorporates both PWB extent and qEEG results, which can be validated and then employed in the community.
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Dr. Ewen conceptualized and designed the study, refined the qEEG metrics, provided clinical interpretation of EEG data, performed supervised steps in the qEEG analysis, performed statistical analyses, and reviewed and revised the final manuscript.
Mr. Tang and Dr. Caffo provided statistical guidance and developed R code for analysis.
Ms. Sebold organized and verified clinical data and performed some statistical analysis.
Dr. Lin provided clinical interpretations of MRI data and revised the final manuscript.
Dr. Srivastava carried out early statistical analyses.
Dr. Srivastava is currently at Harvard University, Boston, MA, USA. Dr. Quain is currently at the University of Michigan, Ann Arbor, MI, USA.
Dr. Gill organized and verified clinical data, performed early statistical analysis, wrote the initial draft of the paper, and revised the final manuscript.
AUTHOR CONTRIBUTIONS
Mr. Adamek and Ms. McAuliffe performed analysis of the EEG data.
Mr. Lakshmanan wrote the MATLAB script to perform analysis of the EEG data.
Dr. Comi conceptualized the initial design of the study and performed all clinical assessments and verification of MRI interpretation from outside sites. She reviewed and revised the final manuscript.
Ms. Smegal organized and verified clinical data, performed some statistical analysis and revised the final manuscript.
Ms. Quain provided original organization of the early EEG database and early analysis of the data.
Dr. Kossoff provided clinical interpretations of EEG data and revised the final manuscript.
ISSN:1388-2457
1872-8952
DOI:10.1016/j.clinph.2021.06.030