Hypothesis: Presymptomatic treatment of Sturge-Weber Syndrome With Aspirin and Antiepileptic Drugs May Delay Seizure Onset

Day, Alyssa M.; Hammill, Adrienne M.; Juhász, Csaba; Pinto, Anna; Roach, E. Steve; McCulloch, Charles E.; Comi, Anne M.; Ball, Karen L.; Fisher, Brian; Koenig, James I.; Lawton, Michael T.; Lo, Warren; Marchuk, Douglas A.; Miles, Daniel; Moses, Marsha A.; Wilfong, Angus A.

doi:10.1016/j.pediatrneurol.2018.04.009

Cited by 41 publications

(32 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“… 6 48 129 Thus, delaying or preventing seizure onset in SWS has become a main treatment goal driving recent efforts to enhance early diagnosis of brain involvement. 9 Standard imaging techniques and assessments result in only about a 25% sensitivity in early imaging 130 resulting in the loss of opportunity to intervene early in many children and slowing efforts to determine the most effective presymptomatic treatment. The proposed quantitative and objective study may improve early diagnosis.…”

Section: Discussion and Desseminationmentioning

confidence: 99%

Study protocol: retrospectively mining multisite clinical data to presymptomatically predict seizure onset for individual patients with Sturge-Weber

et al. 2022

View full text Add to dashboard Cite

IntroductionSecondary analysis of hospital-hosted clinical data can save time and cost compared with prospective clinical trials for neuroimaging biomarker development. We present such a study for Sturge-Weber syndrome (SWS), a rare neurovascular disorder that affects 1 in 20 000–50 000 newborns. Children with SWS are at risk for developing neurocognitive deficit by school age. A critical period for early intervention is before 2 years of age, but early diagnostic and prognostic biomarkers are lacking. We aim to retrospectively mine clinical data for SWS at two national centres to develop presymptomatic biomarkers.Methods and analysisWe will retrospectively collect clinical, MRI and neurocognitive outcome data for patients with SWS who underwent brain MRI before 2 years of age at two national SWS care centres. Expert review of clinical records and MRI quality control will be used to refine the cohort. The merged multisite data will be used to develop algorithms for abnormality detection, lesion-symptom mapping to identify neural substrate and machine learning to predict individual outcomes (presence or absence of seizures) by 2 years of age. Presymptomatic treatment in 0–2 years and before seizure onset may delay or prevent the onset of seizures by 2 years of age, and thereby improve neurocognitive outcomes. The proposed work, if successful, will be one of the largest and most comprehensive multisite databases for the presymptomatic phase of this rare disease.Ethics and disseminationThis study involves human participants and was approved by Boston Children’s Hospital Institutional Review Board: IRB-P00014482 and IRB-P00025916 Johns Hopkins School of Medicine Institutional Review Board: NA_00043846. Participants gave informed consent to participate in the study before taking part. The Institutional Review Boards at Kennedy Krieger Institute and Boston Children’s Hospital approval have been obtained at each site to retrospectively study this data. Results will be disseminated by presentations, publication and sharing of algorithms generated.

show abstract

Section: Discussion and Desseminationmentioning

confidence: 99%

Study protocol: retrospectively mining multisite clinical data to presymptomatically predict seizure onset for individual patients with Sturge-Weber

et al. 2022

View full text Add to dashboard Cite

show abstract

“…Moreover, there have been several studies exploring a potential anti-convulsant action of NSAIDs given individually or in combination with anti-seizure drugs. Several of these studies pointed to a potential additive or synergistic interaction ( Day et al, 2019 ; Srivastava and Gupta, 2001 ; Tandon et al, 2003 ; Zhu et al, 2017 ). This might be partly related to an impact on blood-brain barrier penetration of antiseizure drugs based on an inhibition of efflux transporter induction by NSAIDs ( Potschka, 2010 ).…”

Section: Potential Drug Interactions Between Asms and Antipyretic And Anti-inflammatory Drugs Concomitantly Used In Covid-19 Patientsmentioning

confidence: 99%

Management of COVID-19 in patients with seizures: Mechanisms of action of potential COVID-19 drug treatments and consideration for potential drug-drug interactions with anti-seizure medications

et al. 2021

View full text Add to dashboard Cite

In regard to the global pandemic of COVID-19, it seems that persons with epilepsy (PWE) are not more vulnerable to get infected by SARS-CoV-2, nor are they more susceptible to a critical course of the disease. However, management of acute seizures in patients with COVID-19 as well as management of PWE and COVID-19 needs to consider potential drug-drug interactions between antiseizure drugs and candidate drugs currently assessed as therapeutic options for COVID-19. Repurposing of several licensed and investigational drugs is discussed for therapeutic management of COVID-19. While for none of these approaches, efficacy and tolerability has been confirmed yet in sufficiently powered and controlled clinical studies, testing is ongoing with multiple clinical trials worldwide. Here, we have summarized the possible mechanisms of action of drugs currently considered as potential therapeutic options for COVID-19 management along with possible and confirmed drug-drug interactions that should be considered for a combination of antiseizure drugs and COVID-19 candidate drugs. Our review suggests that potential drug-drug interactions should be taken into account with drugs such as chloroquine/hydroxychloroquine and lopinavir/ritonavir while remdesivir and tocilizumab may be less prone to clinically relevant interactions with ASMs.

show abstract

“…A screening brain MRI may be considered in select children with suspected SWS, for example, when presymptomatic treatment is contemplated. A retrospective study suggested the potential benefit of presymptomatic antiepileptic treatment combined with aspirin, 15 but no prospective studies have been completed. Some children with a particularly high risk for seizures may benefit from presymptomatic treatment, for example, when bilateral SWS is suspected (e.g., extensive bilateral PWBs).…”

Section: Neuroimagingmentioning

confidence: 99%

Consensus Statement for the Management and Treatment of Sturge-Weber Syndrome: Neurology, Neuroimaging, and Ophthalmology Recommendations

Sabeti

Ball

Bhattacharya

et al. 2021

Pediatric Neurology

Self Cite

View full text Add to dashboard Cite

Background: Sturge-Weber syndrome (SWS) is a sporadic, neurocutaneous syndrome involving the skin, brain, and eyes. Because of the variability of the clinical manifestations and the lack of prospective studies, consensus recommendations for management and treatment of SWS have not been published. Objective: This article consolidates the current literature with expert opinion to make recommendations to guide the neuroimaging evaluation and the management of the neurological and ophthalmologic features of SWS. Methods: Thirteen national peer-recognized experts in neurology, radiology, and ophthalmology with experience treating patients with SWS were assembled. Key topics and questions were formulated for each group and included (1) risk stratification, (2) indications for referral, and (3) optimum treatment strategies. An extensive PubMed search was performed of English language articles published in 2008 to 2018, as well as recent studies identified by the expert panel. The panel made clinical practice recommendations. Conclusions: Children with a high-risk facial port-wine birthmark (PWB) should be referred to a pediatric neurologist and a pediatric ophthalmologist for baseline evaluation and periodic follow-up. In newborns and infants with a high-risk PWB and no history of seizures or neurological symptoms, routine screening for brain involvement is not recommended, but brain imaging can be performed in select cases. Routine follow-up neuroimaging is not recommended in children with SWS and stable neurocognitive symptoms.

show abstract

Hypothesis: Presymptomatic treatment of Sturge-Weber Syndrome With Aspirin and Antiepileptic Drugs May Delay Seizure Onset

Cited by 41 publications

References 11 publications

Study protocol: retrospectively mining multisite clinical data to presymptomatically predict seizure onset for individual patients with Sturge-Weber

Study protocol: retrospectively mining multisite clinical data to presymptomatically predict seizure onset for individual patients with Sturge-Weber

Management of COVID-19 in patients with seizures: Mechanisms of action of potential COVID-19 drug treatments and consideration for potential drug-drug interactions with anti-seizure medications

Consensus Statement for the Management and Treatment of Sturge-Weber Syndrome: Neurology, Neuroimaging, and Ophthalmology Recommendations

Contact Info

Product

Resources

About