Cover Image

A case of sturge weber syndrome

REKA M

Abstract


Sturge weber syndrome is a unusual  phakomatosis, multisystem disorder involving Eye, Central nervous system, Skin. Incidence is 1-9 per 100,000.  Primary defect is an insult to  procencephalic and mesencephalic neural crest which give rise to vascular malformation in eye, skin and meninges. This is a case presentation of a 16 year old boy with right divergent squint, episcleral hemangioma and pupil showing relative afferent pupillary defect in the right eye. Right eye intraocular pressure was 38mmHg at the first visit. Gonioscopy showed open angles. Fundus of right eye showed pale disc with dilated vessels. Left eye was normal. Imaging showed calcification in occipital lobe. Patient was treated with antiglaucoma medications. Features were          suggestive of Sturge weber syndrome

 


Full Text:

PDF

References


Berg BO. Neurocutaneous syndromes: Phakomatoses and Allied Conditions. In: Swaiman KF, Ashwal S, editors. Pediatric Neurology Principles and Practice. Mosby Inc. Co 1999; 530-50

Takeoka M, Riviello JJ. Sturge Weber Syndrome. Available From: http:// emedicine.com

Reid DE, Maria BL, Drane WE: Central nervous system perfusion and metabolism abnormalities in the Sturge Weber Syndrome. J Child Neurol 1997 Apr; 12 (3) : 218-22.

Khan AN, Turnbull I, Macdonald S, et al. Sturge Weber Syndrome. Available from: http:// emedicine.com

Arzimanaglou A, Aicardi J. The epilepsy of Sturge Weber Syndrome: Clinical features and treatment in 23 patients. Acta Neurol Scand Supp l992; 140: 18-22.

Pascual Casroviejo I, Diaz Gonzalez C, Garcia Melian RM. Sturge Weber Syndrome: Study of 40 patients. Pediatr Neurol 1993 Jul-Aug; 9(4) : 283-88.

Pinton F, Chiron C, Enjolras O, et al. Early Single Photon Emission Computed Tomography in Sturge Weber Syndrome. Journal of Neurology, Neurosurgery and Psychiatry 1997 Nov; vol. 63 (5) :616-21.

Enjolras O, Riche MC, Merland JJ. Facial Portwine Stains and Sturge Weber Syndrome. Pediatrics 1985 Jul; 76 (1) : 48-51.

Osborn AG. Disorders of Histogenesis: Neurocutaneous syndromes. In: Osborn AG editor Diagnostic Neuroradiology. Mosby Inc. Co. 1994; 74-116.

Benedikt RA, Brown DC, Walker R. Sturge Weber Syndrome: cranial MR imaging with Gd–DTPA. Am Jn Neuroradiol 1993 Mar-Apr; 14 (2) : 409-15.

Bentson JR, Wilson GH, Newton TH. Cerebral venous drainage pattern in Sturge Weber Syndrome. Radiology 1957; 68 : 327.

Chugani HT, Maziotta JC, Phelps ME. Sturge Weber Syndrome : a study of cerebral glucose utilization with positron emission tomography. J Pediatr 1989; 114 : 244-53 .

Thomas Sohl KA, Vaslow DF, Maria BL. Sturge Weber Syndrome: a review. Pediatr Neurol 2004 May; 30 (5) : 303-10.

Rochkind S, Hoffman HJ, Hendrick EB. Sturge Weber Syndrome : natural history and prognosis. J Epilep 1990; 3 (Suppl) : 293.


Refbacks

  • There are currently no refbacks.


Creative Commons License
This work is licensed under a Creative Commons Attribution-NoDerivatives 4.0 International License.

An Initiative of The Tamil Nadu Dr MGR Medical University