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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
1
|
| pubmed:dateCreated |
1995-7-6
|
| pubmed:abstractText |
Data were obtained on 171 individuals with Sturge-Weber syndrome via questionnaire and medical records. The age of the study group ranged from 2 months to 59 years; the median was 8 years. In addition to the facial location of port-wine stains in the areas of the trigeminal dermatomes present in 170 patients, 45% also had extracranial port-wine stains over the torso and/or extremities, and 17% had other vascular or pigmentary lesions. Seizures were present in 80% of all patients (87% of those with bilateral and 71% of those with unilateral port-wine stains); in all but one case, seizures were associated with port-wine stains in V1 alone or V1 and V2 trigeminal dermatomes location. The age of onset of seizures ranged from birth to 23 years; 75% had onset of seizures before 1 year of age; these children had an 83% incidence of developmental and academic problems. Fifty-eight percent showed early developmental delay and required special education classes. The rate of retardation showed a decreasing tendency with increasing age of onset of seizures; of the children without seizures, only 6% had developmental delay and 11% required special education classes. Glaucoma was present in 48% of patients (67% unilateral and 33% bilateral). Of all patients with glaucoma, 92% had port-wine stains in both V1 and V2 dermatomes and 8% only in V1. The laterality of glaucoma did not correspond to the trigeminal distribution of the port-wine stains in all instances. Glaucoma was diagnosed during the 1st year of life in 61% and by 5 years in 72%; one patient did not have onset of symptoms until 38 years. The results of our data can serve as a guide for estimating relative risk figures for seizures, glaucoma, and mental retardation in children with Sturge-Weber syndrome.
|
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
IM
|
| pubmed:status |
MEDLINE
|
| pubmed:month |
Jan
|
| pubmed:issn |
0883-0738
|
| pubmed:author | |
| pubmed:issnType |
Print
|
| pubmed:volume |
10
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
49-58
|
| pubmed:dateRevised |
2011-11-17
|
| pubmed:meshHeading |
pubmed-meshheading:7769179-Adolescent,
pubmed-meshheading:7769179-Adult,
pubmed-meshheading:7769179-Age of Onset,
pubmed-meshheading:7769179-Brain,
pubmed-meshheading:7769179-Child,
pubmed-meshheading:7769179-Child, Preschool,
pubmed-meshheading:7769179-Female,
pubmed-meshheading:7769179-Functional Laterality,
pubmed-meshheading:7769179-Humans,
pubmed-meshheading:7769179-Infant,
pubmed-meshheading:7769179-Intellectual Disability,
pubmed-meshheading:7769179-Male,
pubmed-meshheading:7769179-Middle Aged,
pubmed-meshheading:7769179-Prognosis,
pubmed-meshheading:7769179-Psychomotor Performance,
pubmed-meshheading:7769179-Skin Pigmentation,
pubmed-meshheading:7769179-Sturge-Weber Syndrome,
pubmed-meshheading:7769179-Trigeminal Nerve
|
| pubmed:year |
1995
|
| pubmed:articleTitle |
Sturge-Weber syndrome: age of onset of seizures and glaucoma and the prognosis for affected children.
|
| pubmed:affiliation |
Division of Genetics, University of Colorado School of Medicine, Children's Hospital, Denver 80218-1088, USA.
|
| pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
|