Extent of leptomeningeal capillary malformation is associated with severity of epilepsy in Sturge-Weber syndrome

Sugano H, Iimura Y, Igarashi A, Nakazawa M, Suzuki H, Mitsuhashi T, Nakajima M, Higo T, Ueda T, Nakanishi H, Niijima S, Karagiozov K, Arai H, Extent of leptomeningeal capillary malformation is associated with severity of epilepsy in Sturge-Weber syndrome, Pediatric Neurology (2021), doi: https://doi.org/10.1016/j.pediatrneurol.2020.12.012.

Abstract

Background

Sturge-Weber syndrome (SWS) patients have risks of intractable epilepsy and cognitive decline. We hypothesized that leptomeningeal capillary malformation (LCM) extent related to severity of neurological affection in SWS. This study tested the hypothesis in a cross-sectional study of seizure severity and electroencephalography (EEG) findings and a retrospective cohort study for surgical indications related to extent of LCM. 

Methods

We enrolled 112 patients and classified them according to LCM distribution: (1) Bilateral, (2) Hemispheric, (3) Multilobar, and (5) Single lobe. Seizure onset age, semiology and frequency, and EEG findings were compared. Surgical indications were antiepileptic drug resistance, progressive cerebral atrophy, and cognitive decline, and evaluated in each group by Fisher’s exact test, while predictors for surgery by univariate and multivariate analyses. Therapeutic efficacy was evaluated by the SWS-neurological score (SWS-NS). 

Results

Bilateral and Hemispheric groups had early-onset age (4.0-months and 3.0-months), frequent seizures (88.9% and 80.6% had more than once-a-month), focal-to-bilateral tonic-clonic seizures (88.9% and 74.2%), and status epilepticus (100% and 87.1%). Groups did not differ significantly in EEG findings. Surgical indications were present in 77.8% of Bilateral, 88.1% of Hemispheric, and 46.8% of Multilobar groups. Seizure more than once monthly were a surgical treatment predictor. Seizure sub-score improved significantly postoperatively in Hemispheric and Multilobar groups. Even after the surgical treatment, Bilateral and Hemispheric groups SWS-NSs were higher than those of other groups. 

Conclusion

Our study demonstrated a strong association between extensive LCM and epileptic severity. Surgical intervention improved seizure outcome in SWS patients with large LCMs. (248)