Syndrome de Sturge-Weber

Maruani, Annabel

doi:10.1016/j.lpm.2009.07.030

Cited by 13 publications

(7 citation statements)

References 23 publications

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“…It was first described by Sturge in 1879 in a 6-years-old child with epilepsy, facial hemangioma and glaucoma. Weber reported in 1992 cerebral calcifications in patients presenting the same symptomatology [1]. The bilateral and extensive presentation of the hemangioma and neurological involvement as in our case is unusual.…”

Section: Sirsupporting

confidence: 60%

Overlapping of Sturge Weber syndrome and Klippel Trenaunay syndrome: A new case report

Alouani¹,

Bkiyar²,

Dikhaye³

et al. 2018

Our Dermatol Online

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Section: Sirsupporting

confidence: 60%

Overlapping of Sturge Weber syndrome and Klippel Trenaunay syndrome: A new case report

Alouani¹,

Bkiyar²,

Dikhaye³

et al. 2018

Our Dermatol Online

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“…On estime que 10 % des angiomes plans sur le territoire du V1, avec répercussion ophtalmique, sont liés à une atteinte méningée associée [9]. L'atteinte cérébrale reste très peu probable avec un examen neurologique normal, sans comitialité et avec une IRM normale après l'âge de 1 an [10].…”

Section: Discussionunclassified

Angioma of the face: about two cases including one revealing Sturge Weber and Krabbe syndrome

Tiemtore-Kambou¹,

Ouédraogo²,

Ndé-Ouédraogo³

et al. 2017

Our Dermatol Online

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70How to cite this article: Tiemtoré-Kambou BMA, Ouédraogo NA, Ndé/Ouédraogo NA, Traoré F, Ouédraogo PA, Dao BA, Ouattara B, Zanga M, Diallo O, Cissé R. Angioma plane of the face: About two cases of which one revealing sturge weber and krabbe syndrome. Our Dermatol Online. 2017;8(1):70-74. Submission ABSTRACTSturge Weber and Krabbe syndrome or Sturge Weber Syndrome (SWS) is a rare and sporadic disease. It associates with a variety of degrees a flat facial skin angioma in the V1 territory of the trigeminal, a pial angioma and a choroidal angioma all in the one side. The neurological involvement of epileptic seizures in fact all the gravity. The authors report two cases of angiomas plane of the face. The first case presents a complete association of the 3 signs in a 5 year old child. The second case presents an association of angioma and choroidal involvement without cerebral damage to imaging in a 25-year-old adult. They thus highlight the interest of imaging examinations in particular MRI in the diagnosis through the classification and follow-up of this pathology. Case ReportHow to cite this article: Tiemtoré-Kambou BMA, Ouédraogo NA, Ndé/Ouédraogo NA, Traoré F, Ouédraogo PA, Dao BA, Ouattara B, Zanga M, Diallo O, Cissé R. Angiome plan de la face: À propos de deux cas dont l'un révélant un syndrome de sturge weber et krabbe.

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“…[112] The relatively less reporting of the syndrome in our study is perhaps due to the less incidence of the disease (one in 50,000 live births). [2] Ataxia-telangiectasia (AT) is an autosomal recessive inherited disease caused by the mutational inactivation of the ATM gene.…”

Section: Discussionmentioning

confidence: 99%

Neurocutaneous syndrome: A prospective study

et al. 2011

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Background:Neurocutaneous syndromes (NCS) are a group of genetic disorders that produce a variety of developmental abnormalities of the skin along with an increased risk of neurological complications. Cutaneous manifestations usually appear early in life and progress with time, but neurological features generally present at a later age. There is a paucity of data regarding the evolution of skin lesions and their correlation with the central nervous system involvement in children.Aim:The primary objective was to track the course of skin lesions in various forms of NCS in the pediatric age group. Our secondary aim was to assess whether there was any predictive value of the lesions in relation to the neurological manifestations.Materials and Methods:This prospective longitudinal study was conducted at a tertiary care pediatric dermatology referral clinic of the Institute of Child Health, Kolkata, West Bengal. Children between the age group 0 and 12 years were included in the study on the basis of standard diagnostic criteria for different NCS, during the period from March, 2000 to February, 2004, and each of the enrolled cases were followed up for a duration of six years.Results:The study population comprised of 67 children (35 boys, 32 girls).The mean age of presentation was 33.8±27.8 months (range 10 days to 111 months). The various forms of NCS observed was neurofibromatosis 1(NF1) (n=33), tuberous sclerosis complex (TSC) (n=23), Sturge Weber syndrome (n=6), ataxia telangiectasia (n=2), PHACE syndrome (n=1), incontinentia pigmenti (n=1), and hypomelanosis of Ito (n=1). The presentations were varied, ranging from predominantly cutaneous to primarily neurological, depending on the disease entity and age group concerned. There was a significant increase in the number of café au lait macules (CALMs) with time (P=0.0002) in NF1, unlike that of hypopigmented macules of TSC (P=0.15). Statistically, no relation was documented between the evolution of skin lesions and neurological manifestations in the major groups.Conclusion:As NCS is not an uncommon disease in children, it is always necessary to find out the subtle neurological signs, whenever we observe any case with cutaneous markers suggestive of NCS. In addition, it is a must to do a detailed dermatological examination in a child with central nervous system involvement, in the pediatric population. However, the neurological course cannot be predicted from skin lesions.

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Syndrome de Sturge-Weber

Cited by 13 publications

References 23 publications

Overlapping of Sturge Weber syndrome and Klippel Trenaunay syndrome: A new case report

Overlapping of Sturge Weber syndrome and Klippel Trenaunay syndrome: A new case report

Angioma of the face: about two cases including one revealing Sturge Weber and Krabbe syndrome

Neurocutaneous syndrome: A prospective study

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