Behavioral and academic problems in children with Sturge-Weber syndrome: Differences between children with and without seizures

Raches, Darcy; Hiscock, Merrill; Chapieski, Lynn

doi:10.1016/j.yebeh.2012.06.004

Cited by 25 publications

(15 citation statements)

References 25 publications

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“…Attention problems, mood issues and various cognitive difficulties, depending on the side, region of brain involvement and severity of seizures may contribute significantly to quality of life and academic performance [15**]. Early seizure onset and bilateral brain involvement are both risk factors for cognitive impairment.…”

Section: Treatmentmentioning

confidence: 99%

“…If parents are not properly educated on the presentation of seizures, they may miss them altogether. If seizures continue without being controlled, they can drastically worsen cognitive impairments and neurological injury [15**, 16, 17]. Typically, neurologic status stabilizes by school age and this correlates with a stabilization in the glucose hypometabolism noted on PET imaging [18]; likely this is in part due to the increased seizure threshold in older children and in the opening of collateral deep draining vessels which improve venous outflow and therefore stabilize perfusion to that region.…”

Section: Introductionmentioning

confidence: 99%

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Sturge-Weber Syndrome

Bachur

Comi

2013

Curr Treat Options Neurol

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Section: Treatmentmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Sturge-Weber Syndrome

Bachur

Comi

2013

Curr Treat Options Neurol

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“…Chapieski and colleagues (Chapieski, Friendman, & Lachar, 2000; Raches, Hiscock, & Chapieski, 2012) have found that children and adolescents with SWS and a history of seizures or hemiparesis had lower ratings of parent/teacher reported developmental and intellectual functioning. In addition to parent/teacher reported difficulties, there is an association between lowered intelligence and greater seizure intensity (Kramer et al, 2000), greater seizure frequency (verbal intelligence; Behen et al, 2011), younger age of seizure onset (Behen et al, 2011; Pascual-Astroviejo et al, 2008), longer duration of epilepsy (Behen et al, 2011), and poor response to medication (Pascual-Astroviejo et al, 2008) in children and adolescents with SWS.…”

mentioning

confidence: 99%

Intellectual and adaptive functioning in Sturge-Weber Syndrome

Kavanaugh

Sreenivasan

Bachur

et al. 2015

Child Neuropsychology

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The present study examined the intellectual and adaptive functioning in a sample of children and young adults with Sturge-Weber Syndrome. A total of 80 research participants from a SWS study database underwent full neurological evaluation as part of their participation or concurrent medical care. Twenty-nine of the participants received neuropsychological evaluations. Analyses indicated no significant demographic or neurological differences between those who did and did not receive neuropsychological evaluations. Overall, the neuropsychological evaluation sample displayed significantly lower functioning relative to published normative data across domains of intellectual and adaptive functioning. 32% of the sample displayed impaired performance (standard score ≤ 75) in intellectual functioning and 58% displayed impaired performance in adaptive functioning. Hemiparesis status independently predicted overall adaptive functioning while seizure frequency independently predicted overall intellectual functioning. Younger participants displayed significantly higher (more intact) ratings in adaptive functioning compared to older participants, specifically in overall adaptive functioning, motor skills, and community living skills. A composite measure of neurological status (SWS-NRS) incorporating seizure and hemiparesis status effectively distinguished between individuals with impaired or non-impaired adaptive and intellectual functioning, and showed promise as a screening method for identifying individuals with more involved intellectual and/or adaptive needs.

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“…[42] According to the Sturge-Weber Foundation report, 50% of patients have complete control and 39% of them have partial control of seizures with medications. The results showed that children with SWS plus seizures were similar to the epilepsy group in terms of need to special educational programs, whereas children with SWS and no seizures were similar to the healthy controls.…”

Section: And Intellectual Disabilit Y In Swsmentioning

confidence: 99%

“…The results showed that children with SWS plus seizures were similar to the epilepsy group in terms of need to special educational programs, whereas children with SWS and no seizures were similar to the healthy controls. [42] According to the Sturge-Weber Foundation report, 50% of patients have complete control and 39% of them have partial control of seizures with medications. Patients with partial control of seizures have more incidences of developmental delay and more special educational needs.…”

Section: And Intellectual Disabilit Y In Swsmentioning

confidence: 99%

Potential biological targets for bioassay development in drug discovery of Sturge–Weber syndrome

Mohammadipanah

Salimi

2017

Chem Biol Drug Des

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Sturge-Weber Syndrome (SWS) is a neurocutaneous disease with clinical manifestations including ocular (glaucoma), cutaneous (port-wine birthmark), neurologic (seizures), and vascular problems. Molecular mechanisms of SWS pathogenesis are initiated by the somatic mutation in GNAQ. Therefore, no definite treatments exist for SWS and treatment options only mitigate the intensity of its clinical manifestations. Biological assay design for drug discovery against this syndrome demands comprehensive knowledge on mechanisms which are involved in its pathogenesis. By analysis of the interrelated molecular targets of SWS, some in vitro bioassay systems can be allotted for drug screening against its progression. Development of such platforms of bioassay can bring along the implementation of high-throughput screening of natural or synthetic compounds in drug discovery programs. Regarding the fact that study of molecular targets and their integration in biological assay design can facilitate the process of effective drug discovery; some potential biological targets and their respective biological assay for SWS drug discovery are propounded in this review. For this purpose, some biological targets for SWS drug discovery such as acetylcholinesterase, alkaline phosphatase, GABAergic receptors, Hypoxia-Inducible Factor (HIF)-1α and 2α are suggested.

show abstract

Behavioral and academic problems in children with Sturge-Weber syndrome: Differences between children with and without seizures

Cited by 25 publications

References 25 publications

Sturge-Weber Syndrome

Sturge-Weber Syndrome

Intellectual and adaptive functioning in Sturge-Weber Syndrome

Potential biological targets for bioassay development in drug discovery of Sturge–Weber syndrome

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