Disease relevance of Sturge-Weber Syndrome

• Developmental abnormalities consisted of focal cortical dysplasia (5), heterotopia (2), hamartoma (3), cortical duplication (1), polymicrogyria (1), Sturge-Weber syndrome (1), and tuberous sclerosis (1) [1].
• One patient presented with an atypical form of the Sturge-Weber syndrome, and the other with celiac disease and folic acid deficiency [2].
• We measured local cerebral metabolic rate for glucose (ICMRGIc) using positron emission tomography (PET) in six children with Sturge-Weber syndrome (SWS) and in six neurologically asymptomatic children with facial capillary hemangioma suggestive of SWS [3].
• Neuroimaging studies are valuable in the diagnosis and management of neurofibromatosis, tuberous sclerosis, and Sturge-Weber syndrome [4].
• The cranial computed tomography and magnetic resonance imaging with gadolinium revealed left occipital leptomeningeal angiomatosis with calcifications, suggesting a diagnosis of Sturge-Weber syndrome type III [5].

High impact information on Sturge-Weber Syndrome

• Cases of "atypical Sturge-Weber syndrome" (characterised by serpiginous cerebral calcifications and epilepsy without facial port-wine naevus) should be reviewed, and CD should be ruled out in all cases of epilepsy and cerebral calcifications of unexplained origin [6].
• Case Alex, with Sturge-Weber Syndrome affecting the left hemisphere, failed to develop speech throughout early boyhood, and his comprehension of single words and simple commands remained stagnant at an age equivalent of 3-4 years [7].
• OBJECTIVE: To determine whether the extent and degree of glucose hypometabolism defined by PET correlate with seizure characteristics, cognitive function, and interictal EEG abnormalities in children with unilateral cerebral involvement of Sturge-Weber syndrome (SWS) [8].
• Sturge-Weber syndrome: correlation between clinical course and FDG PET findings [8].
• Technetium-99m-HMPAO SPECT in Sturge-Weber syndrome [9].

Chemical compound and disease context of Sturge-Weber Syndrome

• 5 of 17 children (2 cryptogenic, 2 with severe pre/perinatal brain damage and one with Sturge-Weber syndrome) were classified as responders to high-dose vitamin B6 [10].
• MR imaging of Sturge-Weber syndrome: role of gadopentetate dimeglumine and gradient-echo techniques [11].
• CONCLUSIONS--MRI, especially with gadolinium enhancement, appears to be more sensitive than CT in the detection of neurofibromatosis, tuberous sclerosis, and Sturge-Weber syndrome [4].
• Latanoprost in glaucoma associated with Sturge-Weber syndrome: benefits and side-effects [12].
• Vigabatrin in the Sturge Weber syndrome [13].

Biological context of Sturge-Weber Syndrome

• CONCLUSIONS: Latanoprost may significantly reduce intraocular pressure in selected patients with glaucoma associated with Sturge-Weber syndrome [14].
• Four patients with the Sturge-Weber syndrome were studied using the non-invasive Xenon-133 inhalation technique [15].

Anatomical context of Sturge-Weber Syndrome

• Occipital spike discharges have been seen in young children with visual problems, benign occipital epilepsy of childhood, the Sturge-Weber syndrome, and other symptomatic or structural lesions involving the occipital lobe [16].
• Increased fibronectin expression in sturge-weber syndrome fibroblasts and brain tissue [17].
• Altered blood vessel fibronectin expression in Sturge-Weber syndrome could contribute to abnormal vascular structure and function in this disorder [18].
• Intracranial convolutional or gyriform calcification simulating Sturge-Weber syndrome has been described in association with bacterial and viral intracranial infection as well as irradiation and intrathecal methotrexate for central nervous system leukemia [19].

Gene context of Sturge-Weber Syndrome

• Gyriform or serpentine calcification as revealed by computed tomography (CT) scan is rare and is a characteristic finding of Sturge-Weber syndrome (SWS) and celiac disease (CD) [20].
• Intracranial Tc99m MDP uptake in Sturge-Weber syndrome [21].
• To our knowledge, this combination of Sturge-Weber syndrome, bilateral retinitis pigmentosa, and a dislocated lens has not been reported previously [22].
• Growth hormone deficiency in Sturge-Weber syndrome [23].
• CASE: A 10-year-old boy with Sturge-Weber syndrome and glaucoma in the right eye was studied [24].

Analytical, diagnostic and therapeutic context of Sturge-Weber Syndrome

• Our study is the first to concurrently evaluate structure, perfusion, and glucose metabolism in Sturge-Weber syndrome and to show a mismatch between functional and structural brain imaging in both cerebral hemispheres [25].

References