Disease Information for Sturge-Weber disease

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Clinical Manifestations
Signs & Symptoms
Particular physiognomy/Odd looking kids
Craniofacial Abnormalities/Congenital
Facial hemihypertrophy
Bruit of forehead
Face skin lesions
Port wine Stain Birthmark
Unilateral flame nevus/trigeminal facial zone
Behavior problems
Bruit of head/skull
Headache
Hemianesthesia
Hemianopsia/homonymous/sign
Hemiplegic Gait
Hemisensory defects
Infant Seizures
Mental Deficiency Child
Mental/motor retardation in children/signs
Moro Reflex unilateral Abnormal
Neurological symptoms/signs
Seizures
Seizures/Children/recurrent
Stroke Syndrome Child
Destructive Behavior
Psychomotor retardation
Lateral Asymmetry
Ophthalmoscopic/Fundoscopic exam/abnormal
Clinical Presentation & Variations
Presentation/Multiple deformities newborn (odd look)
Disease Progression
Course/Chronic disorder
Course/Chronic only
Demographics & Risk Factors
Population Group
Child
Infant
Population/Pediatrics population
Family History
Family history/Epilepsy
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Population/Infant
Diagnostic Test Results
CT Scan
CT Scan/Head Brain Abnormality
MRI/Head Cortical atrophy
MRI/Head Venography abnormal
MRI/MRA Head abnormal
X-RAY
Xray/Calcifications intracranial/brain/Skull
Xray/Intracranial solitary calcification/Head
Xray/Multiple intracranial calcifications/Head
Xray/Skull Gyriform calcifications/Tramline
Xray/Unilateral intracranial calcifications/Skull
Xray/Skull abnormality
Xray/Intraorbital calcifications/Head
Associated Diseases & Rule outs
Associated Disease & Complications
Blindness
Blindness in Children
Cerebral AV malformation
Choroid angioma
CNS Angioma
Congenital anomalies
Convulsions (grand mal)
Cutaneous angioma/hemangioma
Facial dysplasia
Glaucoma
Glaucoma, secondary
Hemangioma
Hemihypertrophy
Hemiparesis
Hemiplegia
Leptomeningeal Angiomatosis
Mental retardation
Multiple Congenital Anomalies
Multiple congenital anomalies/Mental retardation
Neurobehavior Problems
Port wine nevus/Nevus flammeus/facial
Seizure disorder (epilepsy)
Stroke/Child
Sturge-Weber disease
Disease Mechanism & Classification
Class
CLASS/Jablonski/NIH Archive Anomalies Database
CLASS/Pediatric disorders (ex)
CLASS/Faces-cranio online database (ex)
CLASS/Facial area manifestation/disorder (ex)
CLASS/Capillary/microcirculation disorder (ex)
CLASS/Cardiovascular (category)
CLASS/Brain/CNS disorder (ex)
CLASS/Neurologic (category)
Pathophysiology
Pathophysiology/Congenital Brain malformation
Pathophysiology/Maternal Chromosome mutation
Pathophysiology/Unilateral effects
Process
PROCESS/Autosomal dominant hereditary disease (ex).
PROCESS/Congenital/developmental (category)
PROCESS/Eponymic (category)
PROCESS/Genetic disorder/Spontaneous mutations/sporadic
PROCESS/Hereditofamilial (category)
PROCESS/INCIDENCE/Rare disease (ex)
PROCESS/Neurocutaneous developmental disorder (ex)
PROCESS/Phakomatosis/congenital (ex)
PROCESS/Congenital CNS disorder (ex)
PROCESS/Dystostosis/craniofacial (ex)
PROCESS/Congenital neoplasm (ex)
Synonyms
Synonym
Angio ocul orbit thalam enceph, angioma capillare et venosum calcificans, Angiomatoses Meningo Oculo Facial, ANGIOMATOSIS ENCEPHALOFACIAL, angiomatosis encephalofacialis, angiomatosis encephalotrigeminal, Angiomatosis Meningo Oculo Facial, angiomatosis meningoulofacialis, Angiomatosis oculo orbital thalamo encephalic syndrome, angiomatosis oculo orbito thalamo encephalic syndrome, Angiomatosis Oculoorbital Thalamic Syndrome, cerebrocutaneous angiomatosis, Cerebrofacial angiomatosis, cutaneocerebral angioma, DIMITRI DISEASE, Dimitri Sturge Weber, ectoneurodermal hamartoma, encephalocraniofacial angiomatosis, Encephalocutan angiomatosis, Encephalocutaneous angiomatosis, encephalofacial angiomatosis, Encephalofacial haemangiomat, Encephalofacial haemangiomatosis, Encephalofacial hemangiomatosis, Encephalofacial Hemangiomatosis Syndrome, Encephalofacial Hemangiomatosis Syndromes, encephalofacial neuroangiomatosis, encephalotrigeminal angiomatosis, Encephalotrigeminal Syndrome, fourth phacomatosis, Haemangiectatic hypertrophy, Hemangiomatosis Syndrome Encephalofacial, Hemangiomatosis Syndromes Encephalofacial, Jahnke syndrome, Kalischer syndrome, Klippel Trenaunay, Klippel Trenaunay Weber syndro, Klippel Trenaunay Weber syndrome, Lawford syndrome, meningeal capillary angiomatosis, Meningo Oculo Facial Angiomatoses, Meningo Oculo Facial Angiomatosis, meningo oculofacial angiomatosis, meningofacial angiomatosis, Meningofacial Angiomatosis Cerebral Calcification Syndrome, Milles' syndrome, Muller syndrome, neuro oculocutaneous angiomatosis, neuroangiomatosis encephalofacialis, neurocutaneous syndrome, neuroectodermal hamartoma, Neuroretinoangiomatoses, Neuroretinoangiomatosis, nevoid amentia, Parkes Weber and Dimitri syndrome, Parkes Weber syndrome, Parkes Weber syndrome (disorder), Phakomatosis Sturge Weber, PKWS, Schirmer syndrome, Schirmer's syndrome, Sturge, STURGE DIS, Sturge disease, Sturge Kalischer Weber Syndrome, Sturge Syndrome, Sturge Weber angiomatosis, Sturge Weber anomalad, Sturge Weber Dimitri Syndrome, Sturge Weber Disease, Sturge Weber Krabbe Syndrome, Sturge Weber Phakomatosis, Sturge Weber sequence, Sturge Weber sequence (disorder), Sturge Weber Syndrome, Sturge Weber syndrome (SWS), Sturge Weber Thoma syndrome, Sturge's Syndrome, SWS, Syndrome Encephalofacial Hemangiomatosis, Syndrome Sturge, Syndrome Sturge Kalischer Weber, Syndrome Sturge Weber, Syndrome Sturge Weber Dimitri, Syndrome Sturge Weber Krabbe, Syndrome Sturge's, Syndromes Encephalofacial Hemangiomatosis, trigemino encephalo angiomatosis, Weber Dimitri syndrome, Weber syndrome, Synonym/Cephalotrigeminal angiomatosis, Synonym/Cutaneous angioma/CNS angioma/Choroid angioma, Synonym/Dimitri's disease, Synonym/Encephalofacial angiomatosis, Synonym/Encephalotrigeminal angiomatosis, Synonym/Fourth phacomatosis, Synonym/Jahnke-Kalishire-Lawford-Muller syndrome, Synonym/Leptomeningeal angiomatosis, Synonym/Meningeal capillary angiomatosis, Synonym/Meningo-oculofacial angiomatosis, Synonym/Parkes Weber-Shirmer syndrome, Synonym/Phakomatosis of Sturge-Weber, Synonym/Sturge-Kalischer-Weber syndrome, Synonym/SWS
Treatment
Drug Therapy - Indication
RX/Anticonvulsants
SX/Hemispherectomy
Definition

A group of neurocutaneous disorders manifested by facial and leptomeningeal angiomas, ipsilateral gyriform calcifications of the cerebral cortex, seizures, development delay, hemiplegia, emotional and behavioral problems, and glaucoma and other ocular disorders; Nevus flammeus on the side of the face ipsilateral to angiomatosis sometimes extends to neck, chest, and back; Angiomatosis may occasionally involve the choroid plexus, thyroid, pituitary gland, lungs, gastrointestinal organs, pancreas, ovaries, and thymus; Correlation between the distribution of the nevus and the course of the trigeminal nerve is responsible for naming the syndrome "trigemino-encephalo-angiomatosis," but later findings found the relationship to be fortuitous; The syndrome frequently occurs in incomplete forms, presenting different combinations of symptoms-----------------------

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External Links Related to Sturge-Weber disease
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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