Extent of Leptomeningeal Capillary Malformation is Associated With Severity of Epilepsy in Sturge-Weber Syndrome
| Autor: | Mika Nakazawa, Shinichi Niijima, Takuma Higo, Hajime Arai, Takumi Mitsuhashi, Madoka Nakajima, Kostadin Karagiozov, Hajime Nakanishi, Yasushi Iimura, Hiroharu Suzuki, Ayuko Igarashi, Tetsuya Ueda, Hidenori Sugano |
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| Rok vydání: | 2021 |
| Předmět: |
Male
Hemangioma Cavernous Central Nervous System Pediatrics medicine.medical_specialty Vascular Malformations Sturge–Weber syndrome Status epilepticus Electroencephalography 03 medical and health sciences Epilepsy symbols.namesake Meninges 0302 clinical medicine Developmental Neuroscience Sturge-Weber Syndrome 030225 pediatrics Humans Medicine Epilepsy surgery Cognitive decline Fisher's exact test Retrospective Studies medicine.diagnostic_test business.industry Patient Acuity Infant Retrospective cohort study medicine.disease Capillaries Cross-Sectional Studies Treatment Outcome Neurology Child Preschool Pediatrics Perinatology and Child Health symbols Female Neurology (clinical) medicine.symptom business 030217 neurology & neurosurgery |
| Zdroj: | Pediatric Neurology. 117:64-71 |
| ISSN: | 0887-8994 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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