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| LETTER TO EDITOR |
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| Year : 2016 | Volume
: 1
| Issue : 1 | Page : 58 |
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Sturge-Weber syndrome
Chhaya Divecha, Milind S Tullu
Department of Pediatrics, Seth G. S. Medical College and K E M Hospital, Parel, Mumbai, Maharashtra, India
| Date of Web Publication | 2-Jun-2016 |
Correspondence Address:
Milind S Tullu
"Sankalp Siddhi", Block No. 1, Ground-Floor, Kher Nagar, Service Road, Bandra (East), Mumbai - 400 051, Maharashtra
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2456-1975.183290
How to cite this article:
Divecha C, Tullu MS. Sturge-Weber syndrome. BLDE Univ J Health Sci 2016;1:58 |
Dear Sir,
A 7-year-old boy presented with repeated left-sided focal convulsions since childhood, requiring frequent admissions and multiple anticonvulsants. He had left-sided weakness and global developmental delay. On physical examination, he had right facial port-wine stain. Neurological examination revealed left upper motor neuron facial palsy and left hemiparesis. In view of intractable left focal seizures despite multiple anticonvulsants, he was mechanically ventilated and treated with midazolam infusion. A skull radiograph [Figure 1] revealed gyriform vascular calcification in the right parietal region. The magnetic resonance imaging of brain [Figure 2] showed right-sided cerebral hemiatrophy with gyriform cortical calcification. On improvement, midazolam infusion was tapered and he was extubated. Tonometry was normal. Sturge-Weber syndrome (encephalotrigeminal angiomatosis) is a rare neurocutaneous syndrome that includes a facial port-wine stain and associated leptomeningeal angiomatosis. It is associated with glaucoma, seizures, developmental delay, and hemispheric symptoms. Treatment includes seizure control and ophthalmic evaluation. | Figure 1: Skull radiographs (frontal and lateral) showing typical gyriform vascular calcification in the right parietal region
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 | Figure 2: The magnetic resonance imaging of the brain showing right-sided cerebral hemiatrophy with gyriform cortical calcification
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Acknowledgment
We would like to thank Dr. Avinash Supe, Director (Medical Education and Major Hospitals) and Dean of Seth G. S. Medical College and K E M Hospital for granting permission to publish this manuscript.
Author contributions
Dr. CD and Dr. MST were equally involved in preparing the manuscript and both are designated as 'First Authors' of this manuscript.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
[Figure 1], [Figure 2]
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