• Users Online: 156
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
LETTER TO EDITOR
Year : 2016  |  Volume : 1  |  Issue : 1  |  Page : 58

Sturge-Weber syndrome


Department of Pediatrics, Seth G. S. Medical College and K E M Hospital, Parel, Mumbai, Maharashtra, India

Date of Web Publication2-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
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2456-1975.183290

Rights and Permissions

How to cite this article:
Divecha C, Tullu MS. Sturge-Weber syndrome. BLDE Univ J Health Sci 2016;1:58

How to cite this URL:
Divecha C, Tullu MS. Sturge-Weber syndrome. BLDE Univ J Health Sci [serial online] 2016 [cited 2019 Jun 24];1:58. Available from: http://www.bldeujournalhs.in/text.asp?2016/1/1/58/183290

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

Click here to view
Figure 2: The magnetic resonance imaging of the brain showing right-sided cerebral hemiatrophy with gyriform cortical calcification

Click here to view


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.


    Figures

  [Figure 1], [Figure 2]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Article Figures

 Article Access Statistics
    Viewed611    
    Printed73    
    Emailed0    
    PDF Downloaded85    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]