Elsevier

Pediatric Neurology

Volume 105, April 2020, Pages 21-26
Pediatric Neurology

Original Article
Seizures and Outcome One Year After Neonatal and Childhood Cerebral Sinovenous Thrombosis

https://doi.org/10.1016/j.pediatrneurol.2019.08.012Get rights and content

Abstract

Background

Pediatric cerebral sinovenous thrombosis is a treatable cause of brain injury, acute symptomatic seizures, and remote epilepsy. Our objective was to prospectively study epilepsy and outcomes in neonates and children one year after cerebral sinovenous thrombosis diagnosis.

Methods

Patients with cerebral sinovenous thrombosis were enrolled prospectively from 21 international sites through the Seizures in Pediatric Stroke Study. Clinical data, including acute symptomatic seizures and cerebral sinovenous thrombosis risk factors, were collected at diagnosis. A neuroradiologist who was unaware of the diagnosis reviewed acute imaging. At one year, outcomes including seizure recurrence, epilepsy diagnosis, antiepileptic drug use, and modified Engel score were collected. Outcomes were assessed using the modified Rankin score and the King's Outcome Scale for Childhood Head Injury.

Results

Twenty-four participants with cerebral sinovenous thrombosis were enrolled (67% male, 21% neonates). Headache was the most common presenting symptom in non-neonates (47%, nine of 19). Nine (37.5%) presented with acute symptomatic seizures. Six (25%; 95% confidence interval, 10% to 47%) developed epilepsy by one-year follow-up. No clinical predictors associated with epilepsy were identified. King's Outcome Scale for Childhood Head Injury and modified Rankin scores at one year were favorable in 71%. Half of the patients who developed epilepsy (three of six) did not have infarcts, hemorrhage, or seizures identified during the acute hospitalization.

Conclusion

Our study provides a prospective estimate that epilepsy occurs in approximately one-quarter of patients by one year after diagnosis of cerebral sinovenous thrombosis. Later epilepsy can develop in the absence of acute seizures or parenchymal injury associated with the acute presentation.

Introduction

Cerebral sinovenous thrombosis (CSVT) is an uncommon but serious cause of acute brain injury, acute symptomatic seizures, and remote epilepsy in children. In the large adult International Study on Cerebral Vein and Dural Sinus Thrombosis, nearly 40% experienced seizures at or before diagnosis.1 Seizures are also reported as a common presenting sign in pediatric CSVT case series and registry data,2, 3, 4, 5 but prospective studies of seizures after neonatal and childhood CSVT have been limited and few studies report epilepsy and neurological outcomes beyond discharge.6 In a retrospective Northern California pediatric stroke cohort that included children with arterial ischemic stroke and CSVT, approximately one-quarter of children with an infarct due to CSVT developed epilepsy by two years after stroke and epilepsy incidence rates were similar in patients with arterial or venous infarcts.7 In prior series, 8% to 38% pediatric patients with CSVT have developed epilepsy after variable lengths of follow-up,2,8,9 suggesting that epilepsy is a frequent sequela. However, prospective, systematically collected data regarding epilepsy frequency and severity after pediatric CSVT are lacking.

Our objective was to prospectively study the outcome of neonates and children one year after a CSVT diagnosis, including epilepsy and standardized scoring of neurological disability. In this descriptive article we report on epilepsy and developmental outcomes in neonates and children who present with acute symptomatic seizures at the time of their CSVT diagnosis.

Section snippets

Methods

The Seizures in Pediatric Stroke (SIPS) Study was a prospective cohort study that enrolled term neonates (birth at ≥ 37 weeks' gestational age to 28 days of life) and children (29 days to 18 years of life) with arterial ischemic stroke or CSVT from 21 international sites between June 2011 and December 2012 as previously described.10,11 All sites obtained informed consent from guardians and study approval from local institutional review boards. Only participants with CSVT were included in this

Results

We identified 24 patients with CSVT from a total of 142 with ischemic stroke enrolled in the SIPS study10 (Fig). Sixteen (67%) were male and five (21%) were neonates. Headache was the most common clinical presentation in non-neonates with CSVT (47%, nine of 19 children). The most common risk factor for childhood CSVT was a chronic disorder, identified in 42% (10 of 24). These included suspected or confirmed genetic syndromes and chronic hematologic conditions or malignancies. Baseline

Discussion

In this prospective study of 24 pediatric patients diagnosed with CSVT, a quarter of patients were diagnosed with epilepsy by 12-month follow-up. This is consistent with previous reported prevalence of epilepsy after CSVT of 8% to 38%.2,7, 8, 9 Previous studies in patients with arterial ischemic stroke demonstrated that those with acute seizures at onset of stroke are at higher risk of developing epilepsy.7,18 Prolonged and recurrent seizures in the acute period also predict increased epilepsy

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  • Cited by (19)

    • Cerebral Sinovenous Thrombosis in Infants and Children: A Practical Approach to Management

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      Although infrequently attributable to the CSVT, mortality is reported, particularly if associated with ICH.1,- –7,11,12,14-19,28,43,44,75 Most cohorts, pediatric and neonatal, show that approximately 1/3-2/3 of children have some neurological abnormality at discharge from hospital and long term (when data was available).1,3-6,9-12,14-19,43,44,75-78 It is however unclear if anticoagulants directly improve long-term clinical outcomes.

    • Neurological and Functional Outcomes after Pediatric Stroke

      2022, Seminars in Pediatric Neurology
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      Predictors of poorer cognitive outcomes include the presence of neurological comorbidities,20,96,98,105 male sex,20,105 older age at CSVT,20 and a need for rehabilitation after stroke.20 Symptomatic seizures are a common presentation for pediatric CSVT, and the rates of subsequent epilepsy are between 18%-25%, significantly higher than the risk of epilepsy in the general population.98,99 Lack of symptomatic seizures during acute pediatric CSVT does not preclude development of epilepsy.99

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    Disclaimers: The authors declare no potential conflict of interest with respect to research, authorship, and/or publication of this article.

    Sources of support: Pediatric Epilepsy Research Foundation (grant 112010-007), Auxilium Foundation, National Institutes of Health (2K12NS001692-11 and KL2TR000143).

    ∗∗

    See Appendix I for Seizures in Pediatric Stroke (SIPS) sites and investigators.

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