Spectrum of Magnetic Resonance Imaging Findings in Sturge Weber Syndrome: A Rare Neurocutaneous Syndrome

Bhavik Unadkat,Bhushita Lakhkar,Rajasbala P Dhande,Shreya Tapadia,Varun Singh

doi:10.9734/jpri/2021/v33i34a31818

Abstract

Sturge Weber syndrome is a type of neurocutaneous disorder also known as meningofacial angiomatosis. It is generally seen in paediatric age group and more commonly effects the males which present with delayed milestones, seizure disorder, loss of vision and other neurological deficits. Clinical suspicion of Sturge weber syndrome should be made on the presence of port wine stain(facial nevus) in a young children. MRI (magnetic resonance imaging) plays a key role in detecting the various spectrum of cortical and vascular defects associated with the syndrome. Clinicians can come to a final diagnosis of Sturge weber syndrome, when the clinical history is supplemented with MRI findings. We in our case report will be discussing the various spectrum of MR findings in this rare neurocutaneous syndrome.

Highlights

Sturge Weber syndrome(SWS) is one of the rare paediatric condition, classified under neurocutaneous disorders having varying clinical presentations ranging from facial nevus to central nervous system manifestations like – mental retardation, seizure disorders, glaucoma, ocular choroidal haemangioma and hemi paresis [1]
The clinical presentation was originally described by Sturge in the year 1879 which included fascial port wine stain, glaucoma and focal seizures which was followed by radiological correlation by Weber in 1922 which suggested ipsilateral brain sclerosis as one of the underlying cause [2,3]
According to Roach et al Sturge weber syndrome can be classified into 3 categoriesType I: representing classic syndrome, having both facial and leptomeningeal angiomas; may or may not have glaucoma, Type II: presents with facial angioma without evidence of intracranial disease; may or may not have glaucoma, Type

Summary

INTRODUCTION

Sturge Weber syndrome(SWS) is one of the rare paediatric condition, classified under neurocutaneous disorders having varying clinical presentations ranging from facial nevus ( known as port wine stain) to central nervous system manifestations like – mental retardation, seizure disorders , glaucoma, ocular choroidal haemangioma and hemi paresis [1]. The clinical presentation was originally described by Sturge in the year 1879 which included fascial port wine stain, glaucoma and focal seizures which was followed by radiological correlation by Weber in 1922 which suggested ipsilateral brain sclerosis (atrophy) as one of the underlying cause [2,3]. Over the years various imaging findings on conventional radiographs, CT , MRI have been described, we in our case report will presenting a classical case of Type 1 Sturge weber syndrome and discussing the various neuroimaging findings

CASE PRESENTATION

DISCUSSION

Findings

CONCLUSION

Sturge-Weber syndrome