The role of adenosine A1 receptor agonist in adenosine augmentation therapy for patients with refractory epilepsy in Sturge-Weber syndrome: An in vitro electrophysiological study.

CONCLUSION: Our results supported the potential of AATs using A1R agonist to be a novel therapy for reducing life burden from patients with refractory epilepsy in SWS, with application to epileptic generation region but not propagation region. PMID: 32208337 [PubMed - as supplied by publisher]
Source: Epilepsy and Behaviour - Category: Neurology Authors: Tags: Epilepsy Behav Source Type: research

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AbstractThis review updates the radiologist on current epilepsy surgery practice in children, with a specific focus on the role of imaging in pre-surgical work-up, current and novel surgical techniques, expected post-surgical imaging appearances and important post-operative complications. A comprehensive review of the current and emerging international practices in paediatric epilepsy surgical planning and post-operative imaging is provided with details on case-based radiological findings. A detailed discussion of the pathophysiology and imaging features of different epileptogenic lesions will not be discussed as this is n...
Source: Neuroradiology - Category: Radiology Source Type: research
Ramesh S Doddamani, Manjari Tripathi, Raghu Samala, Mohit Agarwal, Bhargavi Ramanujan, Sarat P ChandraNeurology India 2020 68(2):270-273 The posterior quadratic epilepsy (PQE) is a form of a multilobar epilepsy, involving the temporal-parietal and occipital lobes. Basically, epilepsies with localized networks to the posterior temporal, posterior parietal, and occipital lobes can benefit from this type of surgery. Gliosis due to perinatal insult and cortical dysplasis and angiomas in Sturge Weber syndrome involving the PQ have often been cited in the literature as the etiology for PQE. However, before considering surgery, ...
Source: Neurology India - Category: Neurology Authors: Source Type: research
CONCLUSIONS: Patients with malformations of cortical development have a higher risk of seizure recurrence, and these malformations comprised the main etiology in the reoperation series. Failure of an initial hemispherotomy usually occurs due to incomplete disconnection and needs to be extensively assessed. Outcomes of reoperation are most often favorable, with acceptable complication rates. PMID: 32234979 [PubMed - in process]
Source: Neurosurgical Focus - Category: Neurosurgery Authors: Tags: Neurosurg Focus Source Type: research
Sturge-Weber syndrome (SWS) is a neurocutaneous disorder associated with epilepsy, glaucoma, cognitive impairments, and a port-wine birthmark. Although individuals with SWS are vulnerable to known risk factors for suicide, including chronic illness and physical differences (port-wine birthmark), frequency of suicidal ideation and attempts in youth and adults with SWS is unknown.
Source: Pediatric Neurology - Category: Neurology Authors: Tags: Research Paper Source Type: research
We present the results of preventive antiepileptic treatment in children with Sturge-Weber syndrome and Tuberous Sclerosis Complex as examples of the possible prevention of epilepsy and epilepsy-associated cognitive impairment in children. PMID: 31166003 [PubMed - indexed for MEDLINE]
Source: Neurologia i Neurochirurgia Polska - Category: Neurology Authors: Tags: Neurol Neurochir Pol Source Type: research
We present the results of preventive antiepileptic treatment in children with Sturge-Weber syndrome and Tuberous Sclerosis Complex as examples of the possible prevention of epilepsy and epilepsy-associated cognitive impairment in children. PMID: 31166003 [PubMed - as supplied by publisher]
Source: Neurologia i Neurochirurgia Polska - Category: Neurology Authors: Tags: Neurol Neurochir Pol Source Type: research
ConclusionsThis study shows good long-term seizure outcomes after hemispherectomy. Seizure freedom has been achieved in the majority of the patients after 3 and 5 years.
Source: Interdisciplinary Neurosurgery - Category: Neurosurgery Source Type: research
The objective of the study was to evaluate clinical characteristics and outcome of hemispherotomy in children and adolescents with hemispheric refractory epilepsy in an Italian cohort of patients. METHODS: We retrospectively evaluated the clinical course and outcome of 92 patients with refractory epilepsy who underwent hemispherotomy in three Italian epilepsy centers between 2006 and 2016. Three different approaches for hemispherotomy were used: parasagittal, modified parasagittal, and lateral. RESULTS: Mean age at epilepsy onset was 1.8 ± 2.51 years, and mean duration of epilepsy prior to surgery wa...
Source: Epilepsy and Behaviour - Category: Neurology Authors: Tags: Epilepsy Behav Source Type: research
Sturge-Weber Syndrome (SWS) is a neurocutaneous disorder caused by a somatic mosaic mutation in GNAQ.1 SWS brain involvement causes seizures, stroke-like episodes and/or neurological deficits such as hemiparesis and intellectual disability. Seventy-five percent of infants with SWS will develop seizures within the first year of life.2 Those with more severe epilepsy experience greater cognitive deficits and intellectual disability.3 There is ictal single-photon emission computed tomography evidence that, in SWS, the seizures themselves may contribute to the brain injury due to an abnormal hemodynamic response.
Source: Pediatric Neurology - Category: Neurology Authors: Tags: Perspectives in Pediatric Neurology Source Type: research
Abstract Sturge-Weber syndrome (SWS) is a neurocutaneous disorder characterized by the combination of a facial naevus flammeus and pial angioma, often associated with learning difficulties and/or epilepsy. Here, we report on the neuropsychological characteristics of a cohort of 92 children with SWS seen at a national referral center between 2002 and 2015. Almost a quarter (24%) had a diagnosis of autism spectrum disorder (ASD), with 45% overall having evidence of social communication difficulties (SCD). Autism spectrum disorder was more commonly seen in those individuals with bilateral angioma (p = 0.021). Sig...
Source: Epilepsy and Behaviour - Category: Neurology Authors: Tags: Epilepsy Behav Source Type: research
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