Proper Therapy Selection Improves Epilepsy Outcomes in Patients With Multilobar Sturge-Weber Syndrome - 18/05/23
, Yasushi Iimura, MD, PhD, Hiroharu Suzuki, MD, PhD, Takumi Mitsuhashi, MD, PhD, Tetsuya Ueda, MD, Kazuki Nishioka, MD, Samantha Tamrakar, MD, PhD, Kostadin Karagiozov, MD, PhD, Madoka Nakajima, MD, PhDAbstract |
Background |
Hemispherectomy is an optimal treatment for patients with Sturge-Weber syndrome (SWS) affecting the whole hemisphere; however, a consensus has not been reached regarding therapeutic choices for those with involvement of two to three lobes. In this study, we compared seizure and cognitive outcomes between medical and surgical treatment groups in patients with multilobar involvement.
Methods |
We evaluated 50 patients with multilobar involvement. Surgical indications included (1) antiepileptic drug (AED)-resistant seizures; (2) developmental delay; and (3) cortical atrophy. Twenty-nine patients were classified in the medical treatment group (MTG), and 21 patients were in the surgical treatment group (STG). Seizure type and frequency, SWS electroencephalography score (SWS-EEGS), and pretherapeutic and posttherapeutic SWS neurological scores (SWS-NS) were compared between groups. Median ages at the initial evaluation of the MTG and STG were 4 and 2 years, and at the final evaluation were 13 and 17 years, respectively.
Results |
The STG had a higher incidence (76.2%) of focal to bilateral tonic-clonic seizures and status epilepticus, although no difference in SWS-EEGS. Seizure and cognitive subcategories of SWS-NS at initial evaluation were worse in the STG (P = 0.025 and P = 0.007). The seizure subcategory in MTG and STG improved after therapy (P = 0.002 and P = 0.001). Cognition was maintained in MTG and improved in STG (P = 0.002). The seizure-free rates in MTG and STG were 58.6% and 85.7%, respectively.
Conclusions |
Appropriate therapeutic choices improved seizure outcomes. Although patients who required surgery had more severe epilepsy and cognitive impairment, surgery improved both.
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 work was supported by the Health and Labor Sciences Research Grants on Rare and Intractable Diseases from the Ministry of Health, Labor and Welfare, Japan (grant number 20FC1039 to H.S.) and Japan Science and Technology-Core Research for Evolutional Science and Technology (JST CREST) (grant number JPMJCR20F1, JPMJCR18A5 to H.S.). |
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| Declarations of interest: Hidenori Sugano reports were provided by Juntendo University. |
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| Data statement: The data used for evaluation in this study are open, and researchers can access our data for further research. |
Vol 143
P. 6-12 - juin 2023 Retour au numéro
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