Neurodevelopmental milestone acquisition following early hemispherotomy in Sturge-Weber syndrome

Abstract

Purpose

To investigate the influence of clinical factors on the acquisition of neurodevelopmental milestones in patients with Sturge-Weber syndrome (SWS) who underwent hemispherotomy.

Methods

We retrospectively analyzed 34 patients with SWS who underwent hemispherotomy at the Epilepsy Center of Juntendo University Hospital between 2006 and 2022. Neurodevelopmental milestones were assessed based on the age at acquisition and interval to acquisition following hemispherotomy. Relationships between five clinical factors—age at surgery, surgery side, history of status epilepticus, presence of contralateral lesions, and Engel classification—and neurodevelopmental milestones were analyzed using Spearman’s rank correlation and multiple linear regression models.

Results

Motor milestones were achieved at 29 and 38 months of age for standing and walking, respectively. Language milestones were achieved at 28 and 41 months of age for words and sentences, respectively. “Age at surgery” was significantly associated with motor milestones (Age_Standing, r = 0.675, p < 0.001; Age_Walking, r = 0.707, p < 0.001) and “Contralateral lesion” was significantly associated with Age_Word (r = 0.641, p < 0.001). Multiple regression models revealed that age at surgery and contralateral lesions were significant predictors of neurodevelopmental milestone acquisition.

Conclusion

Neurodevelopmental milestones were achieved at an average of 29 months for standing, 38 months for walking, 28 months for first words, and 41 months for two-word sentences. Younger age at hemispherotomy and the absence of contralateral lesions were associated with earlier acquisition of neurodevelopmental milestones in patients with SWS.