Outcomes and associations in children with Sturge-Weber syndrome and glaucoma
Sturge-Weber syndrome (SWS) is a rare, neurocutaneous syndrome associated with port-wine stains of the face, glaucoma, leptomeningeal angiomas, seizures, and intellectual disability. Few studies report an overall description of findings and treatment for children with Sturge-Weber glaucoma (SWG), which can be challenging to manage. (Source: Journal of AAPOS)
Source: Journal of AAPOS - August 1, 2021 Category: Opthalmology Authors: Harald Gjerde, Abdelrahman M. Elhusseiny, Deborah VanderVeen Source Type: research

Neurological presentations and cognitive outcome in Sturge-Weber syndrome
CONCLUSIONS: This is the largest reported series documenting detailed developmental profiles of children with SWS, including ID and ASD. PWS(+) shows high rates of ID and language disorder. PWS(-) SWS has a more favourable outcome. Cognitive outcome is contingent on number of affected lobes and bilateral involvement. Epilepsy exerts an additional deleterious effect on language and cognition. A high percentage of children have a history of status epilepticus, with evidence that this impacts language and cognitive outcomes. Acutely acquired neurological deficits did not penalise either. Regular structured clinical and develo...
Source: European Journal of Paediatric Neurology - July 22, 2021 Category: Neurology Authors: Sebastian Powell Tangunu Fosi Jenny Sloneem Christina Hawkins Hanna Richardson Sarah Aylett Source Type: research

Neurological presentations and cognitive outcome in Sturge-Weber syndrome
CONCLUSIONS: This is the largest reported series documenting detailed developmental profiles of children with SWS, including ID and ASD. PWS(+) shows high rates of ID and language disorder. PWS(-) SWS has a more favourable outcome. Cognitive outcome is contingent on number of affected lobes and bilateral involvement. Epilepsy exerts an additional deleterious effect on language and cognition. A high percentage of children have a history of status epilepticus, with evidence that this impacts language and cognitive outcomes. Acutely acquired neurological deficits did not penalise either. Regular structured clinical and develo...
Source: European Journal of Paediatric Neurology - July 22, 2021 Category: Neurology Authors: Sebastian Powell Tangunu Fosi Jenny Sloneem Christina Hawkins Hanna Richardson Sarah Aylett Source Type: research

Neurological presentations and cognitive outcome in Sturge-Weber syndrome
CONCLUSIONS: This is the largest reported series documenting detailed developmental profiles of children with SWS, including ID and ASD. PWS(+) shows high rates of ID and language disorder. PWS(-) SWS has a more favourable outcome. Cognitive outcome is contingent on number of affected lobes and bilateral involvement. Epilepsy exerts an additional deleterious effect on language and cognition. A high percentage of children have a history of status epilepticus, with evidence that this impacts language and cognitive outcomes. Acutely acquired neurological deficits did not penalise either. Regular structured clinical and develo...
Source: European Journal of Paediatric Neurology - July 22, 2021 Category: Neurology Authors: Sebastian Powell Tangunu Fosi Jenny Sloneem Christina Hawkins Hanna Richardson Sarah Aylett Source Type: research

Sturge-Weber Syndrome: An Extensive Cutaneous Presentation
Acta Med Port. 2021 May 2;34(5):387. doi: 10.20344/amp.12671. Epub 2021 May 3.NO ABSTRACTPMID:34253280 | DOI:10.20344/amp.12671 (Source: Acta Medica Portuguesa)
Source: Acta Medica Portuguesa - July 13, 2021 Category: General Medicine Authors: Regina Caldas Maria Guimar ães Olga Ferreira Source Type: research

Total and partial posterior quadrant disconnection for medically refractory epilepsy: a systematic review
1.5T: 1.5 Tesla; 3T: 3 Tesla; ECoG: electrocorticography; EEG: electroencephalography; FCD: focal cortical dysplasia; fMRI: functional magnetic resonance imaging; icEEG: intracranial electroencephalography; ILAE: International League Against Epilepsy; MEG: magnetoencephalography; MRI: magnetic resonance imaging; PET: positron emission tomography; PQD: posterior quadrant disconnection; PRISMA-P: Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols; PVNH: periventricular nodular heterotopia; SEEG: stereo-electroencephalography; SPECT: single-photon emission computed tomography; SWS: Sturge-Weber syndro...
Source: Seizure: European Journal of Epilepsy - May 28, 2021 Category: Neurology Authors: Christopher Markosian, Vincent Dodson, Helen J. Zhang, Rajeshwari S. Mahalingam, Eric B. Geller, Luke D. Tomycz Source Type: research