Extent of Leptomeningeal Capillary Malformation is Associated With Severity of Epilepsy in Sturge-Weber Syndrome - 24/03/21
, Yasushi Iimura, MD, PhD a, Ayuko Igarashi, MD, PhD b, Mika Nakazawa, MD, PhD b, Hiroharu Suzuki, MD a, Takumi Mitsuhashi, MD a, Madoka Nakajima, MD, PhD a, Takuma Higo, MD, PhD a, Tetsuya Ueda, MD a, Hajime Nakanishi, MD, PhD a, Shinichi Niijima, MD, PhD b, Kostadin Karagiozov, MD, PhD a, Hajime Arai, MD, PhD aAbstract |
Background |
Individuals with Sturge-Weber syndrome (SWS) often expereince intractable epilepsy and cognitive decline. We hypothesized that the extent of the leptomeningeal capillary malformation (LCM) may correlate with the severity of neurological impairment due to SWS. We tested the hypothesis in a cross-sectional study of seizure severity and electroencephalographic (EEG) findings and a retrospective cohort study for surgical indications related to the extent of the LCM.
Methods |
We enrolled 112 patients and classified them according to LCM distribution: (1) bilateral, (2) hemispheric, (3) multilobar, and (4) single lobe. Age at seizure onset, seizure semiology and frequency, and EEG findings were compared. Surgical indications were evaluated for each group by Fisher exact test, and predictors for surgery were evaluated by univariate and multivariate analyses. Therapeutic efficacy was evaluated by the SWS-Neurological Score (SWS-NS).
Results |
The bilateral and hemispheric groups had early seizure onset (4.0 months old and 3.0 months old), frequent seizures (88.9% and 80.6% had more than one per month), focal-to-bilateral tonic-clonic seizures (88.9% and 74.2%), and status epilepticus (100% and 87.1%). The groups’ EEG findings did not differ substantially. Surgical indications were present in 77.8% of the bilateral, 88.1% of the hemispheric, and 46.8% of the multilobar groups. Seizure more than once per month was a predictor of surgical treatment. Seizure subscore improved postoperatively in the hemispheric and multilobar groups. Even after surgical treatment, the bilateral and hemispheric groups exhibited higher SWS-NSs than members of the other groups.
Conclusion |
Our study demonstrated a strong association between extensive LCM and epilepsy severity. Surgical intervention improved seizure outcome in patients with SWS with large LCMs.
Le texte complet de cet article est disponible en PDF.Keywords : Sturge-Weber syndrome, Surgical indication, Seizure outcome, Cognitive outcome, Epilepsy surgery
Plan
| Funding: This study was supported by the Health and Labor Sciences Research Grants on rare and intractable diseases from the Ministry of Health, Labor and Welfare, Japan (H29- nanchitou-ippan-010) for the corresponding author and Japan Science and Technology Agency (JST) CREST (Grant No. JPMJCR1784) for the corresponding author. |
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| Conflicts of interest: The authors have no conflicts of interest directly relevant to the content of this article. |
Vol 117
P. 64-71 - avril 2021 Retour au numéro
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