Seizure control after epilepsy surgery in early childhood: A systematic review and meta-analysis
Epilepsy Behav. 2021 Oct 29;125:108369. doi: 10.1016/j.yebeh.2021.108369. Online ahead of print.ABSTRACTOBJECTIVE: This meta-analysis aimed to determine the main factors influencing surgical outcomes in children <3 years old with refractory epilepsy.METHODS: The PubMed and Cochrane database were systematically searched for epilepsy surgery outcomes from December 1, 1991, to March 30, 2021, using the following search terms: "Epilepsy surgery OR Seizure operation" AND "under three years" OR "first three years" OR "early childhood" OR "infancy OR infants." Seizure onset, duration of epilepsy, magnetic resonance imaging fin...
Source: Epilepsy and Behaviour - November 3, 2021 Category: Neurology Authors: Hua Li Shuming Ji Bosi Dong Lei Chen Source Type: research
The Effectiveness of Propranolol in Managing Hemorrhagic Choroidal and Exudative Retinal Detachment following Ahmed Glaucoma Valve Implantation in Sturge-Weber Syndrome: Case Report and Literature Review
Sturge-Weber syndrome (SWS) is a congenital neurological disorder that is characterized by hamartomas involving the skin, brain, and eyes and marked by the presence of the facial port-wine stain, which consequently leads to various ocular complications. Among all ocular comorbidities, glaucoma is the most frequently witnessed in SWS patients with a prevalence of 30% –70%. If glaucoma is refractory to conventional medical management, surgical intervention can be considered. Common complications of glaucoma procedures in SWS are choroidal detachment and suprachoroidal hemorrhage. Moreover, we report a 6-year-old girl, know...
Source: Case Reports in Ophthalmology - October 25, 2021 Category: Opthalmology Source Type: research
Endothelial < em > GNAQ < /em > p.R183Q Increases ANGPT2 (Angiopoietin-2) and Drives Formation of Enlarged Blood Vessels
CONCLUSIONS: Gαq-R183Q, when expressed in ECs, establishes constitutively active PLCβ3 signaling that leads to increased ANGPT2 and a proangiogenic, proinflammatory phenotype. EC-R183Q are sufficient to form enlarged CM-like vessels in mice, and suppression of ANGPT2 prevents the enlargement. Our study provides the first evidence that endothelial Gαq-R183Q is causative for CM and identifies ANGPT2 as a contributor to CM vascular phenotype.PMID:34670408 | DOI:10.1161/ATVBAHA.121.316651 (Source: Arteriosclerosis, Thrombosis and Vascular Biology)
Source: Arteriosclerosis, Thrombosis and Vascular Biology - October 21, 2021 Category: Cardiology Authors: Lan Huang Colette Bichsel Alexis Norris Jeremy Thorpe Jonathan Pevsner Sanda Alexandrescu Anna Pinto David Zurakowski Robin J Kleiman Mustafa Sahin Arin K Greene Joyce Bischoff Source Type: research
Endothelial < em > GNAQ < /em > p.R183Q Increases ANGPT2 (Angiopoietin-2) and Drives Formation of Enlarged Blood Vessels
CONCLUSIONS: Gαq-R183Q, when expressed in ECs, establishes constitutively active PLCβ3 signaling that leads to increased ANGPT2 and a proangiogenic, proinflammatory phenotype. EC-R183Q are sufficient to form enlarged CM-like vessels in mice, and suppression of ANGPT2 prevents the enlargement. Our study provides the first evidence that endothelial Gαq-R183Q is causative for CM and identifies ANGPT2 as a contributor to CM vascular phenotype.PMID:34670408 | DOI:10.1161/ATVBAHA.121.316651 (Source: Arteriosclerosis, Thrombosis and Vascular Biology)
Source: Arteriosclerosis, Thrombosis and Vascular Biology - October 21, 2021 Category: Cardiology Authors: Lan Huang Colette Bichsel Alexis Norris Jeremy Thorpe Jonathan Pevsner Sanda Alexandrescu Anna Pinto David Zurakowski Robin J Kleiman Mustafa Sahin Arin K Greene Joyce Bischoff Source Type: research
Endothelial < em > GNAQ < /em > p.R183Q Increases ANGPT2 (Angiopoietin-2) and Drives Formation of Enlarged Blood Vessels
CONCLUSIONS: Gαq-R183Q, when expressed in ECs, establishes constitutively active PLCβ3 signaling that leads to increased ANGPT2 and a proangiogenic, proinflammatory phenotype. EC-R183Q are sufficient to form enlarged CM-like vessels in mice, and suppression of ANGPT2 prevents the enlargement. Our study provides the first evidence that endothelial Gαq-R183Q is causative for CM and identifies ANGPT2 as a contributor to CM vascular phenotype.PMID:34670408 | DOI:10.1161/ATVBAHA.121.316651 (Source: Arteriosclerosis, Thrombosis and Vascular Biology)
Source: Arteriosclerosis, Thrombosis and Vascular Biology - October 21, 2021 Category: Cardiology Authors: Lan Huang Colette Bichsel Alexis Norris Jeremy Thorpe Jonathan Pevsner Sanda Alexandrescu Anna Pinto David Zurakowski Robin J Kleiman Mustafa Sahin Arin K Greene Joyce Bischoff Source Type: research
Endothelial < em > GNAQ < /em > p.R183Q Increases ANGPT2 (Angiopoietin-2) and Drives Formation of Enlarged Blood Vessels
CONCLUSIONS: Gαq-R183Q, when expressed in ECs, establishes constitutively active PLCβ3 signaling that leads to increased ANGPT2 and a proangiogenic, proinflammatory phenotype. EC-R183Q are sufficient to form enlarged CM-like vessels in mice, and suppression of ANGPT2 prevents the enlargement. Our study provides the first evidence that endothelial Gαq-R183Q is causative for CM and identifies ANGPT2 as a contributor to CM vascular phenotype.PMID:34670408 | DOI:10.1161/ATVBAHA.121.316651 (Source: Arteriosclerosis, Thrombosis and Vascular Biology)
Source: Arteriosclerosis, Thrombosis and Vascular Biology - October 21, 2021 Category: Cardiology Authors: Lan Huang Colette Bichsel Alexis Norris Jeremy Thorpe Jonathan Pevsner Sanda Alexandrescu Anna Pinto David Zurakowski Robin J Kleiman Mustafa Sahin Arin K Greene Joyce Bischoff Source Type: research
Endothelial < em > GNAQ < /em > p.R183Q Increases ANGPT2 (Angiopoietin-2) and Drives Formation of Enlarged Blood Vessels
CONCLUSIONS: Gαq-R183Q, when expressed in ECs, establishes constitutively active PLCβ3 signaling that leads to increased ANGPT2 and a proangiogenic, proinflammatory phenotype. EC-R183Q are sufficient to form enlarged CM-like vessels in mice, and suppression of ANGPT2 prevents the enlargement. Our study provides the first evidence that endothelial Gαq-R183Q is causative for CM and identifies ANGPT2 as a contributor to CM vascular phenotype.PMID:34670408 | DOI:10.1161/ATVBAHA.121.316651 (Source: Arteriosclerosis, Thrombosis and Vascular Biology)
Source: Arteriosclerosis, Thrombosis and Vascular Biology - October 21, 2021 Category: Cardiology Authors: Lan Huang Colette Bichsel Alexis Norris Jeremy Thorpe Jonathan Pevsner Sanda Alexandrescu Anna Pinto David Zurakowski Robin J Kleiman Mustafa Sahin Arin K Greene Joyce Bischoff Source Type: research
Endothelial < em > GNAQ < /em > p.R183Q Increases ANGPT2 (Angiopoietin-2) and Drives Formation of Enlarged Blood Vessels
CONCLUSIONS: Gαq-R183Q, when expressed in ECs, establishes constitutively active PLCβ3 signaling that leads to increased ANGPT2 and a proangiogenic, proinflammatory phenotype. EC-R183Q are sufficient to form enlarged CM-like vessels in mice, and suppression of ANGPT2 prevents the enlargement. Our study provides the first evidence that endothelial Gαq-R183Q is causative for CM and identifies ANGPT2 as a contributor to CM vascular phenotype.PMID:34670408 | DOI:10.1161/ATVBAHA.121.316651 (Source: Arteriosclerosis, Thrombosis and Vascular Biology)
Source: Arteriosclerosis, Thrombosis and Vascular Biology - October 21, 2021 Category: Cardiology Authors: Lan Huang Colette Bichsel Alexis Norris Jeremy Thorpe Jonathan Pevsner Sanda Alexandrescu Anna Pinto David Zurakowski Robin J Kleiman Mustafa Sahin Arin K Greene Joyce Bischoff Source Type: research
Outcomes and lessons learned from two decades ’ experience with glaucoma drainage device implantation for refractory Sturge Weber–associated childhood glaucoma
To evaluate glaucoma drainage device (GDD) implantation for refractory pediatric glaucoma associated with Sturge-Weber syndrome (SWS). (Source: Journal of AAPOS)
Source: Journal of AAPOS - October 13, 2021 Category: Opthalmology Authors: Tanya S. Glaser, Landon C. Meekins, Sharon F. Freedman Tags: Major Article Source Type: research
Another strong argument for the early, aggressive management of seizures to optimize neuro-cognitive outcome in Sturge-Weber syndrome
Eur J Paediatr Neurol. 2021 Sep 3:S1090-3798(21)00157-4. doi: 10.1016/j.ejpn.2021.09.001. Online ahead of print.NO ABSTRACTPMID:34531152 | DOI:10.1016/j.ejpn.2021.09.001 (Source: European Journal of Paediatric Neurology)
Source: European Journal of Paediatric Neurology - September 17, 2021 Category: Neurology Authors: Csaba Juh ász Source Type: research
Sturge Weber Syndrome with Pituitary Macroadenoma in an Adult: An Unusual Association
Tariq A Gojwari, Omair Ashraf Shah, Arshad Hussain, Ajaz Mohiuddin, G Hassan, Mohammad Masood, Imran HamidNeurology India 2021 69(4):1135-1136 (Source: Neurology India)
Source: Neurology India - September 2, 2021 Category: Neurology Authors: Tariq A Gojwari Omair Ashraf Shah Arshad Hussain Ajaz Mohiuddin G Hassan Mohammad Masood Imran Hamid Source Type: research
Sturge Weber Syndrome with Pituitary Macroadenoma in an Adult: An Unusual Association
Tariq A Gojwari, Omair Ashraf Shah, Arshad Hussain, Ajaz Mohiuddin, G Hassan, Mohammad Masood, Imran HamidNeurology India 2021 69(4):1138-1139 (Source: Neurology India)
Source: Neurology India - September 2, 2021 Category: Neurology Authors: Tariq A Gojwari Omair Ashraf Shah Arshad Hussain Ajaz Mohiuddin G Hassan Mohammad Masood Imran Hamid Source Type: research
Quantitative EEG improves prediction of Sturge-Weber syndrome in infants with port-wine birthmark
CONCLUSION: qEEG adds information to risk prediction in infants with facial PWB.SIGNIFICANCE: qEEG can be used to help determine whether to obtain an MRI in the first year of life. The data collected can assist in developing a predictive model risk calculator that incorporates both PWB extent and qEEG results, which can be validated and then employed in the community.PMID:34454271 | DOI:10.1016/j.clinph.2021.06.030 (Source: Clinical Neurophysiology)
Source: Clinical Neurophysiology - August 28, 2021 Category: Neurology Authors: Ryan E Gill Bohao Tang Lindsay Smegal Jack H Adamek Danielle McAuliffe Balaji M Lakshmanan Siddharth Srivastava Angela M Quain Alison J Sebold Doris D M Lin Eric H Kossoff Brian Caffo Anne M Comi Joshua B Ewen Source Type: research
Ocular Manifestations of the Sturge-Weber Syndrome
J Ophthalmic Vis Res. 2021 Jul 29;16(3):415-431. doi: 10.18502/jovr.v16i3.9438. eCollection 2021 Jul-Sep.ABSTRACTSturge-Weber syndrome (SWS) or encephalotrigeminal angiomatosis is a non-inherited congenital disorder characterized by neurologic, skin, and ocular abnormalities. A somatic activating mutation (R183Q) in the GNAQ gene during early embryogenesis has been recently recognized as the etiology of vascular abnormalities in SWS. Approximately, half of the patients with SWS manifest ocular involvement including glaucoma as the most common ocular abnormality followed by choroidal hemangioma (CH). The underlying pathophy...
Source: Journal of Ophthalmic and Vision Research - August 16, 2021 Category: Opthalmology Authors: Kiana Hassanpour Ramin Nourinia Ebrahim Gerami Ghavam Mahmoudi Hamed Esfandiari Source Type: research
