A Spectrum of Unusual Neuroimaging Findings in Patients with Suspected Sturge-Weber Syndrome

Adams, Matthew; Aylett, Sarah; Squier, Waney; Chong, WK

doi:10.3174/ajnr.a1350

Cited by 39 publications

(49 citation statements)

References 25 publications

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“…Two studies were excluded from analysis; one included only individuals with confirmed leptomeningeal angiomatosis on MRI and the other was a single‐case report . Definitions of high‐risk PWS differed between studies . Dutkiewicz and colleagues and Waelchli and colleagues classified hemifacial, median and forehead PWS phenotypes as high‐risk, whereas Piram and colleagues and Adams and colleagues used old definitions based on involvement of the first division of the trigeminal nerve.…”

Section: Results Of the Literature Reviewmentioning

confidence: 99%

“…The presence of leptomeningeal angiomatosis was considered a direct feature of SWS (typically involving the posterior parietal or occipital cortex ipsilateral to the PWS), although a spectrum of indirect findings were deemed suggestive (Table ). Dutkiewicz and colleagues and Adams and colleagues defined direct and indirect MRI features of SWS. Subjects were classified as having SWS based on the presence of leptomeningeal angiomatosis on MRI or indirect MRI signs with seizures or other neurodevelopmental disorder .…”

Section: Results Of the Literature Reviewmentioning

confidence: 99%

See 1 more Smart Citation

Screening for Sturge‐Weber syndrome: A state‐of‐the‐art review

Zallmann

Leventer

Mackay

et al. 2017

Pediatric Dermatology

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Infants with a high-risk distribution of port-wine stains are commonly screened for Sturge-Weber syndrome using brain magnetic resonance imaging. There is no consensus about which port-wine stain phenotypes to screen, optimal timing, screening sensitivity, or whether presymptomatic diagnosis improves neurodevelopmental outcomes. This state-of-the-art review examines the evidence in favor of screening for Sturge-Weber syndrome, based on its effect on neurodevelopmental outcomes, against the risks and limitations of screening magnetic resonance imaging and electroencephalography. A literature search of PubMed/MEDLINE was conducted between January 2005 and May 2017 using key search terms. Relevant articles published in English were reviewed; 34 articles meeting the search criteria were analyzed according to the following outcome measures: neurodevelopmental outcome benefit of screening, diagnostic yield, financial costs, procedural risks, and limitations of screening magnetic resonance imaging and electroencephalography. There is no evidence that a presymptomatic Sturge-Weber syndrome diagnosis with magnetic resonance imaging results in better neurodevelopmental outcomes. The utility of electroencephalographic screening is also unestablished. In Sturge-Weber syndrome, neurodevelopmental outcomes depend on prompt recognition of neurologic red flags and early seizure control. Small numbers and a lack of prospective randomized controlled trials limit these findings. For infants with port-wine stain involving skin derived from the frontonasal placode (forehead and hemifacial phenotypes), we recommend early referral to a pediatric neurologist for parental education, counselling, and monitoring for neurologic red flags and seizures and consideration of electroencephalography regardless of whether magnetic resonance imaging is performed or its findings. K E Y W O R D Selectroencephalography, epilepsy, magnetic resonance imaging, neurodevelopmental outcomes, port-wine stain, screening, Sturge-Weber syndrome

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Section: Results Of the Literature Reviewmentioning

confidence: 99%

Section: Results Of the Literature Reviewmentioning

confidence: 99%

Screening for Sturge‐Weber syndrome: A state‐of‐the‐art review

Zallmann

Leventer

Mackay

et al. 2017

Pediatric Dermatology

View full text Add to dashboard Cite

show abstract

“…Cet angiome est souvent situé dans la région pariéto-occipitale et est généralement homolatéral à l'angiome cutané, même s'il peut être bilatéral ou controlatéral [10]. Les autres anomalies pouvant être observées sont des calcifications corticales, un élargissement des plexus choroïdes, une absence de drainage veineux superficiel et une atrophie corticale [8,11]. Toutes ces anomalies sont généralement précoces, mais ne sont pas toujours visibles à quelques mois de vie.…”

Section: Imagerie Cérébraleunclassified

Syndrome de Sturge-Weber

Maruani

2010

La Presse Médicale

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“…SWS'de serebellar atrofi sık görülen bulgular arasında bulunmamaktadır. 15 Atrofiye rağmen hastamızın nörolojik muayenesi migren tarzı baş ağrısı dışında tamamen normal bulunmuştur. SWS'de migrenin mekanizması, epilepsi ve vasküler malformasyonlardakine benzer özelliktedir.…”

Section: Resi̇munclassified

Sturge-Weber Syndrome with Different Intracranial Pathologies: Case Report

Demir¹,

Yazıcı²

2012

Turkiye Klinikleri J Med Sci

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882turge-Weber sendromu (SWS) "ensefalotrigeminal anjiyomatozis" diye de adlandırılan nörokutanöz bir hastalıktır. Genellikle leptomeninksleri ve yüz derisinde trigeminal sinirin oftalmik (V1) ve maksiller (V2) dağılım alanlarını etkiler. Kutanöz anjiyom porto şarabı nevüs olarak adlandırılır. A An na ah ht ta ar r K Ke el li im me el le er r: : Sturge-Weber sendromu; beyin neoplazileri; araknoid kistler A AB BS ST TR RA AC CT T Sturge-Weber Syndrome (SWS) is usually characterized with port-wine facial nevus, leptomeningeal angiomatosis and glaucoma. Although neurologic findings vary according to the location and size of the cerebral pathology, epilepsy and hemiparesis are the most common findings. In this study, we presented a 31-year-old male patient who had a port-wine facial nevus. Leptomeningeal angiomatosis and glaucoma that often accompanied Sturge-Weber Syndrome were not detected in our patient. His neurologic examination was normal. Magnetic resonance imaging revealed a mass in the cerebellopontine angle and an arachnoid cyst on the left temporal region.. Additionally extensive cerebral and cerebellar atrophy was detected without significant vascular lesion. With these findings, we considered the patient a variant of Sturge-Weber Syndrome Type II. Although SWS variants were reported in the literature, no reports are available presenting the coexistance of the syndrome with such intracranial pathologies.

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A Spectrum of Unusual Neuroimaging Findings in Patients with Suspected Sturge-Weber Syndrome

Cited by 39 publications

References 25 publications

Screening for Sturge‐Weber syndrome: A state‐of‐the‐art review

Screening for Sturge‐Weber syndrome: A state‐of‐the‐art review

Syndrome de Sturge-Weber

Sturge-Weber Syndrome with Different Intracranial Pathologies: Case Report

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