Suicide Screening in Sturge-Weber Syndrome: An Important Issue in Need of Further Study

Sebold, Alison J.; Ahmed, Amelia S.; Ryan, Taylor C.; Cohen, Bernard A.; Jampel, Henry D.; Suskauer, Stacy J.; Zabel, T. Andrew; Comi, Anne M.; Rybczynski, Suzanne

doi:10.1016/j.pediatrneurol.2020.03.013

Cited by 8 publications

(5 citation statements)

References 26 publications

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“…This replicates the finding by Harmon et al in which males had worse Ped NQoL cognitive scores 12 . These important findings may inform the increased risk of positive suicide screening in males with SWS found by Sebold et al 34 The mechanism of sex differences in SWS is unknown.…”

Section: Discussionsupporting

confidence: 87%

Longitudinal prospective study of Sturge–Weber syndrome urine angiogenic factors and neurological outcome

Kimbrell,

McKenney,

Yeom

et al. 2024

Annals of the Child Neurology Society

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ObjectiveThis study identified biomarkers of neurological outcome in Sturge‐Weber syndrome (SWS) via urine angiogenic factors and captured longitudinally derived natural history data within an SWS cohort.MethodsThis longitudinal, prospective, multicentered study of 61 people with SWS aged 0.4–55 years reports port‐wine birthmark score, Neuroscore, Neuro‐Quality of Life, and urine angiogenic factors over a two‐year period.ResultsCognitive Neuroscore worsened over time for children aged 0–2 years. Male sex was associated with worsening Cognitive Function Neuroscore during the study. Age of seizure onset before 2 years was strongly associated with worse Neuroscore. Children with SWS had low Neuro‐Quality of Life related to cognitive function. Seizure severity, male sex, and earlier age of seizure onset were associated with worse Neuro‐Quality of Life in school‐aged children. Children with SWS have elevated basic fibroblast growth factor in their urine compared with controls, whereas higher vascular endothelial growth factor was associated with better Neuroscore.InterpretationThis study is the first multicenter, prospective, and longitudinal study of people with SWS. It identifies significant clinical prognostic factors such as age of seizure onset and male sex, informs symptom progression over time by age group, and suggests that further study of angiogenic mechanisms and potential biomarkers are needed.

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Section: Discussionsupporting

confidence: 87%

Longitudinal prospective study of Sturge–Weber syndrome urine angiogenic factors and neurological outcome

Kimbrell,

McKenney,

Yeom

et al. 2024

Annals of the Child Neurology Society

View full text Add to dashboard Cite

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“…The study showed that patients with SWS are more susceptible to suicidal ideations than patients with some other neurological abnormalities. Thus, families who have patients suffering from SWS should be counselled in detail regarding the risks of suicidal ideations 8 .The reported case of 38 year old female patient manifested all the symptoms attributed to SWS. However, she had massive macroglossia which is not commonly seen.…”

Section: Discussionmentioning

confidence: 99%

Sturge-Weber syndrome with massive macroglossia and anterior neck space infection- a case report and review of literature

Iftikhar,

Osama Khalid,

Uzma Ghori

2024

J Pak Med Assoc

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Sturge- Weber syndrome (SWS), is a rare neuro-cutaneous angiomatosis which affects male and females alike. The clinical manifestations include angiomas, haemangiomas of the lips, tongue and palatine region. The oral manifestations are usually unilateral and are susceptible to bleed. Patients can also present with macroglossia and maxillary bone hypertrophy which can lead to malocclusion of the oral cavity. Food accumulation due to occlusion can cause growth of bacteria which can intensify infections and can cause gingival hyperplasia. A case of a middle-aged 39 year old female was reported in the Ziauddin Hospital, Karachi on 2nd of February,2022 with the presenting complaints of intermittent fever and drowsiness for 10 days. On examination she had massive tongue enlargement, drooling, malocclusion, difficulty in eating and breathing. ---Continue

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“…Adding clinical variables to neuroimaging is important. Males are associated with worse cognitive outcomes, and with a greater risk of suicidality in SWS 134 135. Whether gender impacts the early evolution of SWS as manifested in neuroimaging remains to be studied.…”

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

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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.

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Suicide Screening in Sturge-Weber Syndrome: An Important Issue in Need of Further Study

Cited by 8 publications

References 26 publications

Longitudinal prospective study of Sturge–Weber syndrome urine angiogenic factors and neurological outcome

Longitudinal prospective study of Sturge–Weber syndrome urine angiogenic factors and neurological outcome

Sturge-Weber syndrome with massive macroglossia and anterior neck space infection- a case report and review of literature

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

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