Literature DB >> 28978576

Bilateral intracranial calcifications with bilateral facial cutaneous naevus: Sturge Weber syndrome.

Muhammad Arif Saeed1, Kiran Hilal1, Prem Chand2.   

Abstract

Sturge Weber syndrome also known as mother spot disease belongs to a group of disorders called phakomatoses (neurocutaneous syndromes involving the central nervous and cutaneous systems). It is a congenital disorder of the vasculature of the meninges, brain, face and eyes. Clinically, it usually presents with seizures and other neurological complications, including mental retardation, contralateral hemiparesis and glaucoma. Its incidence is estimated at one case in 20 000-50 000 persons with equal frequency in boys and girls. We present a case of 18-months-old boy was brought by parents with history of seizures since birth resulting in fall 1 day back. Physical examination showed bilateral port-wine stain on face. CT head examination revealed bilateral intracranial calcification. This is a highly unusual presentation of Sturge Weber syndrome. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Entities:  

Keywords:  neurology; paediatrics; paediatrics (drugs and medicines); radiology

Mesh:

Year:  2017        PMID: 28978576      PMCID: PMC5652342          DOI: 10.1136/bcr-2017-219985

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  5 in total

1.  Diagnosis of Sturge-Weber syndrome: comparison of the efficacy of CT and MR imaging in 14 cases.

Authors:  L Martí-Bonmatí; F Menor; C Poyatos; H Cortina
Journal:  AJR Am J Roentgenol       Date:  1992-04       Impact factor: 3.959

Review 2.  Sturge-Weber syndrome: from the past to the present.

Authors:  Annapurna Sudarsanam; Simone L Ardern-Holmes
Journal:  Eur J Paediatr Neurol       Date:  2013-11-07       Impact factor: 3.140

3.  Sturge-Weber syndrome with bilateral intracranial calcification.

Authors:  E Boltshauser; J Wilson; R D Hoare
Journal:  J Neurol Neurosurg Psychiatry       Date:  1976-05       Impact factor: 10.154

Review 4.  Sturge-Weber Syndrome: A Review.

Authors:  E Higueros; E Roe; E Granell; E Baselga
Journal:  Actas Dermosifiliogr       Date:  2017-01-23

5.  Location of port-wine stains and the likelihood of ophthalmic and/or central nervous system complications.

Authors:  B Tallman; O T Tan; J G Morelli; J Piepenbrink; T J Stafford; S Trainor; W L Weston
Journal:  Pediatrics       Date:  1991-03       Impact factor: 7.124

  5 in total

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