scholarly journals Infraslow Status Epilepticus: A new form of subclinical status epilepticus recorded in a child with Sturge Weber Syndrome

2015 ◽  
Vol 42 (S1) ◽  
pp. S27-S28
Author(s):  
LE Bello-Espinosa
Keyword(s):  
Status Epilepticus ◽  
Structural Changes ◽  
Continuous Eeg ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
New Form ◽  
Acute Confusion ◽  
Structural Epilepsy ◽  
Focal Status Epilepticus ◽  
Patients With Epilepsy

Background: Analysis of infraslow EEG activity (ISA) has shown potential in the evaluation of patients with epilepsy and in differentiating between focal and generalized epilepsies. The purpose of this report is to present a girl with Sturge-Weber Syndrome (SWS) who was identified to have infraslow status epilepticus (ISSE), which successfully resolved after Midazolam administration Methods: The continuous EEG recording of a 5-yr-old girl with known Structural Epilepsy due to Sturge-Weber is presented. The patient presented to the ED with acute confusion, eye deviation and right hemiparesis similar to two previous admissions. ltm-eeg showed diffuse slowing. DWI obtained within 72-hr showed no ischemic changes, analysis of the EEG nfraslow (ISA) activity was undertaken using LFF 0.01 AND HFF of 0.1 Hz respectively. Results: Continuous subclinical unilateral rhythmic ictal ISA was identified. This was only evident in the left hemisphere which correlated with the structural changes due to SWS. A trial of continuous IV midazolam resulted in immediate resolution of the contralateral hemiparesis and encephalopathy. Conclusion: Continuous prolonged rhythmic ictal Infraslow activity (ISA) can cause super-refractory subclinical focal status epilepticus. This has not previously reported, and we propose this should be called Infraslow status epilepticus (ISSE). ISA analysis should be performed in all patients with unexplained subclinical status epilepticus.

scholarly journals Focal cortical dysplasia type IIa underlying epileptogenesis in patients with epilepsy associated with Sturge-Weber syndrome

Epilepsia ◽  
2012 ◽  
Vol 53 (11) ◽  
pp. e184-e188 ◽  
Author(s):  
Nobuya Murakami ◽  
Takato Morioka ◽  
Satoshi O. Suzuki ◽  
Kimiaki Hashiguchi ◽  
Toshiyuki Amano ◽  
...  
Keyword(s):  
Focal Cortical Dysplasia ◽  
Cortical Dysplasia ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Patients With Epilepsy

scholarly journals Continuous Visual Focal Status Epilepticus as the Primary Presentation of NMDA-R and GAD65-R Autoimmune Epilepsy

2020 ◽  
Vol 11 ◽  
Author(s):  
Elma M. Paredes-Aragón ◽  
Héctor E. Valdéz-Ruvalcaba ◽  
Andrea Santos-Peyret ◽  
Marcela Cisneros-Otero ◽  
Raúl Medina-Rioja ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Early Identification ◽  
Acid Decarboxylase ◽  
Visual Aura ◽  
Epilepsia Partialis Continua ◽  
Autoimmune Epilepsy ◽  
Immune Mediated ◽  
Focal Status Epilepticus ◽  
Patients With Epilepsy ◽  
Primary Presentation

Epilepsia partialis continua (EPC) has changed in its clinical and pathophysiological definition throughout time. Several etiologies have been described in addition to classic causes of EPC. The following case depicts a young woman who had a peculiar onset of epilepsy with a continuous visual aura becoming a form of chronic recurrent and non-progressive EPC. The patient was initially misdiagnosed as a non-neurological entity (assumed psychiatric in origin), but finally, an immune-mediated epilepsy was diagnosed, and EEG showed focal status epilepticus during evolution. Once the diagnosis was achieved and immune treatment was established, the patient is seizure free. Early identification of an immune basis in patients with epilepsy is important because immunotherapy can reverse the epileptogenic process and reduce the risk of chronic epilepsy. To date, this is the only case reported with EPC manifesting as a continuous visual aura associated with antiglutamic acid decarboxylase 65 (anti-GAD65) and anti-N-methyl-d-aspartate (anti-NMDA) antibodies.

P38.5 Case report – Status epilepticus and Sturge–Weber Syndrome

2006 ◽  
Vol 117 ◽  
pp. 1-2
Author(s):  
A. Arsovska ◽  
A. Popovski ◽  
Z. Arsovski
Keyword(s):  
Case Report ◽  
Status Epilepticus ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

Neonatal status epilepticus controlled with levetiracetam at Sturge Weber syndrome

2013 ◽  
Vol 35 (4) ◽  
pp. 367-371 ◽  
Author(s):  
Sema Tanriverdi ◽  
Demet Terek ◽  
Ozge Altun Koroglu ◽  
Mehmet Yalaz ◽  
Hasan Tekgul ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

Status epilepticus and venous infarction in Sturge-Weber syndrome

1998 ◽  
Vol 14 (12) ◽  
pp. 693-696 ◽  
Author(s):  
S. C. Coley ◽  
J. Britton ◽  
A. Clarke
Keyword(s):  
Status Epilepticus ◽  
Venous Infarction ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

scholarly journals D.02 Predictive factors for epilepsy in pediatric patients with Sturge Weber Syndrome

2016 ◽  
Vol 43 (S2) ◽  
pp. S13-S13
Author(s):  
ML Kaseka ◽  
JY Bitton ◽  
P Major
Keyword(s):  
Venous Drainage ◽  
P Value ◽  
Significant Difference ◽  
Weber Syndrome ◽  
Medical Photography ◽  
Patients At Risk ◽  
Sturge Weber Syndrome ◽  
D 02 ◽  
Patients With Epilepsy

Background: Sturge Weber Syndrome (SWS) patients at risk of epilepsy are often not identified before their first seizure which leads to unnecessary follow up of many patients with facial angioma. Methods: The medical photography database of our institution has been reviewed to identify SWS patients followed between 1993 and 2013. Patients with isolated glaucoma were compared to patients with epilepsy regarding the location of the facial angioma, the presence of asymmetrical background activity on EEG done prior epilepsy onset and cerebral imaging. Logistical regression tests and a p-value of 0.05 were used. Results: 21 patients with SWS have been identified. No significant difference was noted when patients were compared based on the laterality of the lesion (p=0.169), or the location of the facial angioma (p = 0.314 to 0.999). Only 2 epileptic patients had digital EEG done prior the onset of epilepsy and only 2 patients with glaucoma had digital EEG done during their follow up. No significant difference was noted between EEG background activities in the two groups (p= 0.514). The presence of venous drainage anomalies (VDA) predicted (p = 0.004) the onset of epilepsy. Conclusions: Cerebral VDA increases the risk of epilepsy in SWS patients. Since they can be detected at birth, they might guide the management.

scholarly journals Structural Abnormalities on MRI in Cases with Epilepsy

2020 ◽  
Vol 8 (2) ◽  
pp. 18-22
Author(s):  
Gaurav Kaushik ◽  
Madan Manmohan
Keyword(s):  
Vascular Malformations ◽  
Age Groups ◽  
The Elderly ◽  
Common Finding ◽  
Weber Syndrome ◽  
History Of ◽  
Sturge Weber Syndrome ◽  
History Of Epilepsy ◽  
Mri Study ◽  
Patients With Epilepsy

Introduction: Many treatable anatomical abnormalities of the brain can cause seizures. The present study was done to determine the role of MRI for evaluation of patients with epilepsy. Subjects and Methods: Patients who presented to our hospital with history of epilepsy. i.e two or more episodes of unproved seizures 24 hours apart, underwent MRI study to assess for abnormalities. Patients of either gender and all age groups who were referred to our department for MRI study were included. Results: Generalized tonic clonic seizures were present in 86% of the population and rest had partial seizures. Using MRI, we arrived at a diagnosis as a cause of seizure in 51.3% of the patients and rest of     the 48.7% had a normal MRI study. The most common pathology detected on MRI was infarct with gliosis (24.7%). Less common pathology detected were neurocysticercosis (6.7%), brain atrophy (5.3%), tuberculoma (3.3%), venous thrombosis (2.7%), developmental malformations (2.7%), glioma (1.3%), cavernoma (1.3%), tuberous sclerosis (1.3%), meningioma (0.7%), cerebral abscess (0.7%) and Sturge Weber syndrome (0.7%). Abnormality on MRI was not significantly associated with gender or type of seizure. MR abnormality was observed maximum in patients between 1 to 30 years (30 out of 70 patients i.e. 42.8%) while all the elderly cases showed abnormal MRI, with infract with gliosis being the most common finding. Conclusions : MR imaging should be the first investigation of choice in epileptic syndrome, cerebrovascular disease with seizure, developmental cortical malformations, and vascular malformations.

scholarly journals Abnormal neurovascular coupling during status epilepticus migrainosus in Sturge-Weber syndrome

Neurology ◽  
2016 ◽  
Vol 88 (2) ◽  
pp. 209-211 ◽  
Author(s):  
Moksh Sethi ◽  
Magdalena A. Kowalczyk ◽  
Linda J. Dalic ◽  
John S. Archer ◽  
Graeme D. Jackson
Keyword(s):  
Status Epilepticus ◽  
Neurovascular Coupling ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome
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