Seizure and developmental outcomes following epilepsy surgery for children with Sturge-Weber syndrome

Jonathan Cookson1, Hanna Richardson2, Martin Tisdall3

  • 1UCL Great Ormond Street Institute of Child Health, London, UK; Department of Neurodisability, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.

Insights

Epilepsy surgery for Sturge-Weber syndrome (SWS) offers good seizure control, particularly in younger patients. While most children maintain or improve cognitive and language skills, motor function can be affected.

Area of Science:

  • Neuroscience
  • Pediatric Neurology
  • Neurosurgery

Background:

  • Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder frequently associated with epilepsy in children.
  • Epilepsy surgery is a potential treatment, but outcomes regarding seizure control and neurodevelopment are not fully understood.

Purpose of the Study:

  • To evaluate seizure control and neurodevelopmental outcomes in children with SWS undergoing epilepsy surgery.
  • To analyze the impact of surgery on cognition, language, motor function, and social communication.

Main Methods:

  • Retrospective review of case notes for children with SWS who underwent epilepsy surgery.
  • Analysis of standardized developmental assessments for cognition, language, and motor function.

Main Results:

  • 77.2% of patients achieved good seizure control (Engel 1 outcome).
  • Older age at surgery was linked to poorer seizure outcomes.
  • Cognitive and language trajectories mostly stabilized or improved; motor skills showed mixed results, with fine motor skills declining in 60%.

Conclusions:

  • Epilepsy surgery provides effective seizure control for SWS patients.
  • Younger age at surgery appears to correlate with better outcomes.
  • Further multicenter studies are necessary to identify factors influencing seizure and developmental outcomes.
Abstract

Related Concept Videos

Epilepsy and Seizures: Overview01:24

Epilepsy and Seizures: Overview

Epilepsy is a chronic neurological disease marked by recurrent, unpredictable seizures. These seizures are caused by abnormal electrical discharges in the brain, leading to behavior, sensation, or consciousness alterations. They can also cause transient impairment of awareness, interfering with daily activities.
Various factors can trigger epilepsy, including genetic factors, brain damage, metabolic causes, and unknown etiology. Diagnosis of epilepsy involves electroencephalography (EEG), which...
Seizures: Classification01:13

Seizures: Classification

Epilepsy is primarily characterized by unpredictable seizures, either provoked by an identifiable factor, such as injury or illness, or unprovoked, occurring spontaneously without apparent cause.
Seizures are typically classified into two main categories: focal and generalized seizures.
Focal Seizures
Focal seizures originate from specific regions of the brain. These seizures are further sub-classified into two types:
Seizures l: Introduction01:20

Seizures l: Introduction

Understanding seizures and epilepsy relies on key definitions that help in recognizing, classifying, and managing these disorders. These definitions provide a framework for recognizing, classifying, and managing seizure disorders.DefinitionsA seizure is a sudden, abnormal burst of electrical activity in the brain that can cause changes in awareness, movement, sensation, or behavior, depending on the area involved. Epilepsy is a chronic condition characterized by recurrent, unprovoked seizures,...
Epilepsy ll: Types01:22

Epilepsy ll: Types

Recurrent seizures, stemming from abnormal electrical activity in the brain, are the defining characteristic of epilepsy, a chronic neurological condition. Because seizure features vary greatly, epilepsy is classified using two systems: by seizure type and by epilepsy syndromes. These classifications enable clinicians to describe seizure patterns and select suitable treatment strategies.I. Classification by Seizure Type1. Focal EpilepsyFocal epilepsy begins in one hemisphere of the brain.
Seizures ll: Types01:19

Seizures ll: Types

Seizures are sudden bursts of abnormal electrical discharge in the brain that interfere with normal function. They are commonly divided into three groups: focal seizures, generalized seizures, and other types that do not fit neatly into either category.Focal SeizuresFocal seizures begin in a single brain region. When awareness is preserved, they are called focal aware seizures and may cause sensations such as tingling, unusual smells, or flashing lights. When awareness is impaired, they are...