Surgery Can Get Favorable Outcome in Atypical Sturge-Weber Syndrome With Intractable Epilepsy

Liang, Chuandong; Liu, Na; Wu, Jiang; Tang, Yufei; Guo, Tao; Yue, Xiang-yong; Kang, Jin-sheng; Li, Wenling; Zhao, Wenqing

doi:10.1097/scs.0000000000001340

Cited by 2 publications

(11 citation statements)

References 8 publications

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“…2,9,10 It is thought to be caused by abnormal persistence of embryonic venous plexus in close proximity to ectoderm that was destined to form venous drainage of occipital and parietal region of brain as well as facial skin. 7,10 The low flow angiomata in Sturge-weber syndrome are at risk of thrombosis and calcification which eventually leads to ischemia and gliosis of surrounding nervous tissue and atrophy. 2,8 These patients can benefit from daily aspirin to prevent thrombosis in low flow angioma.…”

Section: Discussionmentioning

confidence: 99%

“…11 Common symptoms are seizures (75-90%), intellectual disability and developmental delay (50-75%), hemiplegia (40-45%), headache (40-60%), glaucoma (30-70%), hemianopsia (40-45%), and hemiparesis (25-60%). 10,11 It is commonly diagnosed in neonates and rarely found later in life. There are only few case reports describing the diagnosis of this syndrome in the 5 th and 6 th decade of life 9 Patients with seizure freedom more than 6 months at a time are considered to have a good seizure control, which is noted in 60-70% of patients with anti-seizure medications.…”

Section: Sectionmentioning

confidence: 99%

“…3 Patients with drug-resistant epilepsy in SWS have undergone surgical options like lobectomy, hemispherectomy, and corpus callosotomy. 10,11 Patients who undergo early surgery have the potential to achieve seizure freedom and significant reduction postoperatively. 10 Presurgical work up requires neuropsychology assessment for memory evaluation and language lateralization.…”

Section: Sectionmentioning

confidence: 99%

“…10,11 Patients who undergo early surgery have the potential to achieve seizure freedom and significant reduction postoperatively. 10 Presurgical work up requires neuropsychology assessment for memory evaluation and language lateralization. Patients with drugresistant epilepsy benefit from further discussions at a comprehensive epilepsy conference to seek opinion from different providers including neuroradiologist, neuropsychologist as well as the neurosurgeon.…”

Section: Sectionmentioning

confidence: 99%

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Clinical Reasoning: Drug Resistant Epilepsy in a 61-Year-Old Man With Abnormal MRI Brain Findings and Management With Vagal Nerve Stimulator

Mankad¹,

Lavingia

2023

Neurology

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A 66-year-old man with seizures that started at 61 years eventually developed drug-resistant epilepsy and was managed with medications and vagal nerve stimulation. The patient had a convulsive event at age 61, followed by recurrent events of confusion and speech arrest lasting 30-120 seconds. He underwent gadolinium enhanced MRI brain and angiogram which revealed pial enhancement in the right occipital, parietal, and posterior temporal regions with subcortical atrophy. CSF findings were unremarkable. Continuous video EEG showed electroclinical correlation for his episodes of confusion and speech arrest with recurrent brief runs of rhythmic delta from the right temporal region with evolution and spread to the entire right hemisphere. The patient tried multiple antiseizure medications including valproic acid, topiramate, phenytoin, carbamazepine, levetiracetam, brivaracetam, lamotrigine without success. He was eventually put on a combination of lacosamide, zonisamide, clonazepam, and primidone, which helped to certain extent, but the patient continued to have daily episodes and 10-12 electroclinical seizures noted on a follow-up 24-hour ambulatory EEG. Follow-up MRI brain with contrast confirmed the diagnosis. Phase II intracranial monitoring for surgical management was offered to the patient which he deferred due to risks. Vagal nerve stimulator (VNS) was also offered as a palliative therapy to which the patient agreed. Gradual titration of VNS settings over 1 year helped to achieve seizure freedom. Presentation of focal seizure with this type of atypical etiology is rare. Typically surgical management is used to achieve seizure freedom in this condition; successful management with VNS has not been reported so far.

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Section: Discussionmentioning

confidence: 99%

Section: Sectionmentioning

confidence: 99%

Section: Sectionmentioning

confidence: 99%

Section: Sectionmentioning

confidence: 99%

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Clinical Reasoning: Drug Resistant Epilepsy in a 61-Year-Old Man With Abnormal MRI Brain Findings and Management With Vagal Nerve Stimulator

Mankad¹,

Lavingia

2023

Neurology

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“…In cases of drug-resistant epilepsy, surgical treatment can be offered. 5,6,[16][17][18][19][20][21][22][23] Traditionally, hemispherectomy has been the surgery of choice for SWS; however, focal lesionectomy of the affected cortex is advocated as well. 10 The aim of this case-based systematic review and meta-analysis was to provide an overview of the literature regarding focal lesionectomy as a surgical treatment option in SWS, with a focus on seizure outcome, motor and cognitive development, and morbidity.…”

mentioning

confidence: 99%

Focal lesionectomy as surgical treatment of epilepsy in patients with Sturge-Weber syndrome: a case-based systematic review and meta-analysis

Frank

Greuter

Dill

et al. 2022

Neurosurgical Focus

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OBJECTIVE Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder presenting mostly with a facial port-wine stain and leptomeningeal angiomatosis. More than 85% of the patients are affected by epilepsy by the age of 2 years. Seizure and symptom control is the focus of SWS treatment, since no causal therapy exists yet. For pharmacologically intractable epilepsy, surgery is a treatment option. The aim of this systematic review and meta-analysis was to provide an overview of the literature regarding lesionectomy in SWS with a focus on seizure outcome, complications, and motor and cognitive development. METHODS The PubMed and Embase databases were searched using a systematic search strategy to identify studies on SWS from their inception until 2021. Two independent researchers assessed the studies for inclusion and quality. Outcome measures were seizure outcome, postoperative complications, and motor and cognitive development. Thereafter, a systematic review was conducted, and a meta-analysis was performed for all included cohort studies. Risk of bias was assessed using the Newcastle-Ottawa Scale. Forest plots have been generated for all outcomes; risk ratio was used for pooled outcomes. A p value < 0.05 was considered as statistically significant. RESULTS After removal of duplicates, the authors screened 439 articles, of which 9 articles with 150 patients were included. Our case and 5 case reports and 4 retrospective cohort studies were included for systematic review. The latter 4 studies qualified for the meta-analysis. In these 4 articles, 144 patients received surgical treatment: 81 (56%) underwent focal lesionectomy and 63 (44%) hemispherectomy. Pooled outcome analysis for postoperative favorable seizure outcome showed a nonsignificant difference between lesionectomy and hemispherectomy (69.2% vs 87.3%; RR 0.73, 95% CI 0.50–1.08; t = −2.56, p = 0.08). Lesionectomy showed a significantly lower rate for developmental delay and postoperative hemiparesis in comparison with hemispherectomy (29.8% vs 76.3%; RR 0.41, 95% CI 0.28–0.59; z = −4.77, p < 0.0001 and 18.1% vs 100%; RR 0.11, 95% CI 0.06–0.21; z = −6.58, p < 0.0001, respectively). CONCLUSIONS Based on the limited literature available, lesionectomy leads to a nonsignificant lower seizure control rate, while postoperative developmental or motor deficits are significantly lower compared with hemispherectomy. Therefore, focal lesionectomy remains a valid alternative to hemispherectomy in SWS with a clearly localized epileptogenic area; however, individual case-based decisions in a specialized multidisciplinary team are of paramount importance.

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Surgery Can Get Favorable Outcome in Atypical Sturge-Weber Syndrome With Intractable Epilepsy

Cited by 2 publications

References 8 publications

Clinical Reasoning: Drug Resistant Epilepsy in a 61-Year-Old Man With Abnormal MRI Brain Findings and Management With Vagal Nerve Stimulator

Clinical Reasoning: Drug Resistant Epilepsy in a 61-Year-Old Man With Abnormal MRI Brain Findings and Management With Vagal Nerve Stimulator

Focal lesionectomy as surgical treatment of epilepsy in patients with Sturge-Weber syndrome: a case-based systematic review and meta-analysis

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