Cognitive impairment patterns and associated clinical and imaging characteristics in young patients with Sturge-Weber syndrome

Halah Z Keramane; Michael E Behen; Aimee F Luat; Csaba Juhász

doi:10.1016/j.braindev.2026.104554

Cognitive impairment patterns and associated clinical and imaging characteristics in young patients with Sturge-Weber syndrome

Brain Dev. 2026 Jun 5;48(4):104554. doi: 10.1016/j.braindev.2026.104554. Online ahead of print.

Authors

Halah Z Keramane¹, Michael E Behen², Aimee F Luat³, Csaba Juhász⁴

Affiliations

¹ Wayne State University School of Medicine, Detroit, MI, USA; Translational Imaging Laboratory, University Health Center, Detroit, MI, USA.
² Translational Imaging Laboratory, University Health Center, Detroit, MI, USA; Department of Pediatrics, Wayne State University School of Medicine, Detroit, MI, USA; Department of Neurology, Wayne State University School of Medicine, Detroit, MI, USA.
³ Department of Pediatrics, Wayne State University School of Medicine, Detroit, MI, USA; Department of Neurology, Wayne State University School of Medicine, Detroit, MI, USA; Department of Pediatrics, Central Michigan University, Mount Pleasant, MI, USA; Children's Hospital of Michigan, Detroit, MI, USA.
⁴ Translational Imaging Laboratory, University Health Center, Detroit, MI, USA; Department of Pediatrics, Wayne State University School of Medicine, Detroit, MI, USA; Department of Neurology, Wayne State University School of Medicine, Detroit, MI, USA; Children's Hospital of Michigan, Detroit, MI, USA. Electronic address: csaba.juhasz@wayne.edu.

Abstract

Background: Most patients with Sturge-Weber syndrome (SWS) have unilateral brain involvement associated with a wide range of neurocognitive outcomes.

Aims: To evaluate cognitive deficit patterns in young patients with unilateral SWS and identify clinical and imaging variables associated with the various cognitive deficit patterns.

Methods: Forty-six young patients with SWS were stratified by the side of brain involvement and assigned to four cognitive groups, based on formal neuropsychology evaluation: 1-no deficit, 2-traditional deficit (verbal deficit in left, non-verbal in right SWS), 3-reorganized with crowding (e.g., only non-verbal deficit in left-hemispheric cases), and 4-global (verbal and non-verbal) deficit. Age, seizure variables, motor functions, and the extent of brain magnetic resonance imaging abnormalities were compared among the cognitive groups.

Results: The reorganized/crowding pattern was seen only in patients with left SWS (6/20, 30%), most of whom were young with extensive abnormalities. Traditional cognitive deficit was seen mostly in right SWS (7/26, 27%). Global deficit was equally common (35%) in left- and right SWS and was associated with extensive hemispheric abnormalities, early seizure onset, and severe motor deficits. In multivariate analysis, extensive calcifications and severe motor deficits were independently associated with global cognitive deficit.

Conclusions: Early, extensive left hemispheric abnormalities can be associated with preserved verbal but impaired non-verbal functions consistent with right-hemispheric reorganization and crowding. However, more than one-third of both left and right-hemispheric patients may develop a global cognitive impairment. This severe cognitive outcome is associated with extensive hemispheric calcification and severe motor deficit.

Keywords: Calcification; Cognitive functions; Epilepsy; Magnetic resonance imaging; Motor functions; Sturge-Weber syndrome.